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PGY2-General Dermatology Flashcards

1
Q

List 6 clinical variants psoriasis

A
  • Plaque
  • Pustular-multiple subtypes
  • Guttate
  • Erythrodermic
  • Special locations-scalp, inverse, nail, oral
  • Psoriatic arthritis: 5 subtypes
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2
Q

List 5 variants pustular psoriasis

A
  • Generalized pustular psoriasis VEAL
    o Von Zombusch
    o Exanthematic
    o Annular of lapier
    o Localized
    -Gestational/Impetigo herpetiformis
  • Acrodermatitis continua of Hallopeau
  • Acrodermatitis repens of Crocker
  • Pustular psoriasis of palms and soles

VEALGAAP

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3
Q

List psoriasis triggers

A

PITTED NAILS

  • Psychogenic stress
  • Infection (e.g., streptococcus)
  • Trauma/koebner
  • Terrible habits (e.g., EtOH and smoking)
  • Endocrine (e.g., pregnancy, hypocalcemia)
  • Drugs= NAILS (see below)
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4
Q

List 5 systemic associations with psoriasis

A

a. Metabolic dz
b. Diabetes
c. Obesity
d. IBD
e. PG
f. RA
g. Celiac

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5
Q

List 10 causes drug induced psoriasis

A
  • NSAIDS-e.g. indomethacin
  • Anti-malarial -CQ/HQ
  • Anti-hypertensive (ACEi, betablockers)
  • Infliximab and other anti-TNFs
  • Interferon
  • Imiquimod
  • Lithium
  • Steroids withdrawl

Others:
* Quinidine
* Gemfibrozil
* Clonidine
* Rapid taper of cyclosporine
* GM-CSF/G-CSF
* SSKI
* Bupropion/Wellbutrin

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6
Q

What are the variants of PsA

A

SODAS:
Spondylitis + sacroiliitis (5%): often HLA-B27 + (w/ IBD, uveitis)

Asymmetric Oligoarthritis + Mono (70%)

DIPs (OA-like, 5%): exclusively DIPs, classic, but uncommon

Arthritis Mutilans: (5%, least common)

Symmetric RA-like (MCPs, PIPs, 15%): sym polyarthritis of small-med joints

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7
Q

What 3 psoriasis sites in a patient are high risk for arthritis

A
  • Scalp
  • Nails
  • Gluteal cleft
  • Severe psoriasis
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8
Q

Name 10 histological signs in psoriasis

A
  • Regular acanthosis
  • Confluent parakeratosis
  • Munroe’s micro abscesses (stratum corneum)
  • Spongiform pustules of Kojog (stratum spinosum)
  • Squirting papillae (neutrophils discharge from papillary dermal tips)
  • Thinning supra-papillary plates
  • Dilated and tortuous capillaries in dermal papillae
  • Elongation dermal papillae
  • Superficial perivascular infiltrate w/ lymphocytes and macrophages
  • Decreased or absent granular layer
  • Elongated and squared off rete ridges
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9
Q

What does PASI stand for? What min and max score?

A

a. Psoriasis Area and Severity Index
b. Erythema, scale, thickness/induration
c. Minimum: 0, Max: 72

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10
Q

Name 4 cytokines elevated in psoriasis

A
  • IL-17
  • IL-23
  • IL-22
  • TNF
  • Also: IFN, IL-2, Il-1, IL-6
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11
Q

Name 5 classes of medications used for psoriasis

A
  • PDE-4 inhibitor (Apremilast)
  • Retinoids (Acitretin)
  • Immunosuppressants (MTX, Cyclosporine)
  • Biologics (TNF, 12/23, 23, 17)
  • JAK inhibitor (Tofacitinib)
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12
Q

Name 4 systemic associations with prp

A

celiac sprue
myositis
MG
hiv

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13
Q

According to the Griffith classification, what are the 5 variants of pityriasis rubra? What is the 6th additional variant?

A

Type I- Classic adult (scalp erythema, keratitis follicular papules on red base, salmon plaques, islands sparing, orange-red waxy keratoderma)
Type II- Atypical adult (itchyosiform leg lesions, eczema and lamellate coarse ppt)
Type III-Juvenile classic
Type IV-Juvenile circumscribed (knees, elbows follicular pap)
Type V-Juvenile atypical (sclerodermoid hands and feet)
Type VI-HIV associated
Type VII-Facial discoid dermatosis

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14
Q

What are 6 morphological variants of Pityriasis Rosea?

A

Gigantea – confined to herald patch
Urticarial
Vesicular
Pustular
Inverse
Purpuric
EM-like

GUV PIPE:

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15
Q

What are 5 medications known to cause drug induced PR?

A

KFC BAGS TOH

Ketotifen
Flagyl
Clonidine

Beta blockers, bismuth, barbiturates, BCG vaccine
ACEi, Aresnic, accurate
Gold
Salvarsan

TNF in
omeprazole
HCTZ

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16
Q

Name top 5 causes erythroderma per Scott Walsh

A

Dermatitic- AD>contact>SD>actinic
Psoriasis
drug rxn
MF
idiopathic

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17
Q

List 10 causes of Erythroderma (please ensure that the first 5 are listed most common to least common)

A
  • Dermatitis
  • Psoriasis
  • Drugs
  • CTCL
  • Idiopathic
  • Seborrheic dermatitis
  • Stasis dermatitis with autosensitization
  • PRP
  • Ichthyosis
  • Bullous dermatoses-e.g. Bullous pemphigoid
  • GVHD
  • AI-CTD: e.g. Dermatomyositis
  • Infestation-scabies
  • Paraneoplastic
  • Chronic actinic dermatitis
  • Papuloerythroderma of ofuji
  • Mastocytosis
  • PER SCOTT WALSH
    o Dermatitic -24% (AD>contact>seb>actinic)
    o Psoriasis -20%
    o Drug rxn -19%
    o CTCL -8%
    o Idiopathic
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18
Q

List 5 variants pustular psoriasis

A
  • Generalized pustular psoriasis of von Zumbusch
  • Exanthematous pustular psoriasis
  • Annular pustular psoriasis of LaPierre
  • Localized pustular psoriasis
  • Gestational (Impetigo herpetiformis)
  • Acrodermatitis continua of Hallopeau
  • Acrodermatitis repens of Crocker
  • Pustulosis palmaris et plantaris
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19
Q

Name which findings in psoriasis are due to what parts of nail bed?
Salmon patch
Oil drop sign
Splinter hemmorhages
Pitting
Beaus lines
Leukonycia
Erythema of lunula
Distal onycholysis
Sublingual hyperkeratosis

A
  • Salmon patch/oil drop – NB
  • Splinter hemorrhages – NB
  • Distal onycholysis – NB
  • Subungual hyperkeratosis – NB or distal matrix
  • Pitting – PNM
  • Beau’s lines – PNM
  • Leukonychia – Distal nail matrix
  • Erythema of lunula – DNM

Key:
* PNM - proximal nail matrix
* DNM - distal nail matrix
* NB - nail bed
* H - hyponychium

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20
Q

What are the HLA associations for each of the following? HLA Cw6, HLA B27, HLA B13/B17

A
  • HLA-Cw6: early onset psoriasis, increased severity, fmhx
    o Increased risk Caucasians (13x), Japanese (25x)
  • HLA-B27: reactive arthritis, sacroiliitis, pustular PsO
  • HLA-B13/HLA-B17: erythrodermic PsO
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21
Q

From epidemiologic studies, what is the risk of psoriasis in an offspring if:
1 parent
both parents
1 sibiling
No parent/sibling

A
  • One parent is affected? 14%
  • Both parents are affected? 41%
  • One sibling is affected? 6%
  • No parent or sibling is affected? 2%
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22
Q

Define the following:
Auspitz sign
Woronoff ring
Koebner phenomenon

A

Woronoff: hypopigmented ring surrounding individual psoriasis lesions possibly due to inhibition of prostaglandin synthesis by treatments.

Koebner: traumatic induction of psoriasis on non-lesional skin.

The Auspitz sign: focal bleeding points that appear when the scale is removed due to dilated capillaries and thinned suprapapillary plates

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23
Q

Name 5 systemic associations w/ LP

A

Hep C
Dental amalgams
Drug
GVHD
paraneoplastic

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24
Q

List 10 variants lichen Planus

A

Annular
Actinic
Atrophic
Bullous
Eruptive/exanthematous
Hypertrophic
Linear
Lichen planopilaris
Lichen Plans pigmentosus
LP pemphigoides
Ulcerative
Oral
Inverse
Nail
Vulvovaginal
DLE/LP overalp

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25
List 8 classes of drugs known to cause lichenoid drug eruptions. Name
Anti 6 PANG (6 Antis then PANG) Anti-hypertensives: ACEi, BB, methyl dopa, HCTZ Anti-malarial: HCQ, Chloroquine, Quinacrine, quinidine Anti-depressants: Amitryptiline, lithium Anti-fungal: ketoconazole Anti-TNFs: Etanercept, infliximab Anti-cancer: Pembrolizumab, Nivolumab,, trosine kinase inhibitors e.g. matinib PANG Penicllamine Allopurinol NSAIDS Gold salts
26
Name 10 features seen on histology for LP
Orthokeratosis Hyperkeratosis Irregular acanthosis Saw tooth rete ridges Wedge shaped hypergranulosis Hypereosinophilia of keratinocytes at Malphigian layer Lymphocytic band like infiltrate DEJ Basal layer vacuolar degeneration Colloid bodies papillary dermis Civatte bodies basal layer Pigment incontinence Subepidermal clefts between epidermis and dermis: Max-Joseph spaces
27
Name 4 associations with PLCA and PLEVA
Hodgkins, B cell lymphoma HIV CTCL
28
Name 10 porokeratos variants
a. DSAP b. DSP (non-actinic) c. Porokeratosis palmaris et plantaris disseminate d. Porokeratosis plantaris discrete e. Punctate porokeratosis f. Linear porokeratosis g. Porokeratosis of Mibelli h. Porokeratosis psychotropica i. Porokeratoma j. Pruritic papular porokeratosis k. Porokeratotic adnexal ostial nevus
29
Name 4 mutations in porokeratosis
a. MVK-mevalonate kinase, PMVK phosphomevalonate kinase, MVD mevalonate diphosphate decarboxylase, FDPS farnesyl diphosphate synthase. b. *End up with deficiency in cholesterol c. Treat with topical statin (cholesterol inhibitor) and cholesterol
30
What is the highest risk porokeratosis for scc
linear porokeratosis
31
18) Name 5 conditions caused by malessizia furfur/sympodialis in yeast form and 1 in mycelium form
Yeast: seb derm head neck AD pityrosporum folliculitis CARP Neonatal cephalic pustolosis Mycelium PV
32
19) EDV: Name the 2 HPV subtypes, the 2 clinical presentations, the genetics predispositions
EVER 1 and 2- regulate zinc transport in nucleus HPV 5/8 PV like, flat wart like Risk SCC in PV-like if photo exposed
33
Name 2 associations w; nekams
heme malig IDA
34
Name 4 presentations/additional features seen in nekams
Seb derm like lesions Aphthous ulcers PPK Blepharitis/conjuctivitis *alopecia in children
35
How is drug induced lichen planus different from classic lichen planus
Older age in lichenoid drug eruption (65 vs. 50) More generalized distribution on the trunk, often spares classic LP sites (forearms, wrists, genitals) Whickam’s striae less common Larger lesions, more eczematous, psoriasiform or PR-like morphology Mucous membranes usually spared Frequently photo distributed
36
What are 5 morphological variants of systemic ACD to nickel?
Eyelid dermatitis Widespread nummular dermatitis Hand dermatitis Popmpholyx Intertriginal dermatitis Anogenital baboon syndrome Erythroderma a. Symmetrical eyelid dermatitis b. Hand dermatitis c. Pompholyx (dishydrotic eczema) d. Dermatitis in skin folds e. Anogenital baboon syndrome (peri-anal/genital) f. Widespread nummular dermatitis g. Erythroderma h. Generalized mac pap i. Recall dermatitis j. Flare up genital site k. Peri-orbital/perioral
37
List 5 allergens that can lead to systemic ACD.
Metals: nickel Plants: Poison ivy (Mangos/cashew exposure) Balsam of Peru (sodas, citrus foods, spices like cloves, vanilla, toothpaste) Sesqueterpene lactone -->chamomile tea Meds: Topical steroids (systemic steroids) Neomycin-->aminoglycosides, Quinolones Ampicillin Ethylenediamine hydrochloride --> aminophyline Foods: Sorbic acid--> sorbic acid Formaldehyde --> aspartame
38
6 allergens that can lead to contact anaphylaxis
BAN PCR Bacitracin Ammonium peruslfate Neomycin PPD Chlorehexidine Rubber-latex
39
Describe the grading system for patch testing
i. +/- macular erythema/doubtful reaction ii. + weak, non-vesicular with erythema, papules and infiltration iii. ++ strong vesicular reaction with erythema, infiltration and papules iv. +++ spreading bullous reaction v. IR= irritant vi. – is negative rxn
40
If a patient is unable to perform patch testing, what other alternatives exist and how is it performed?
Repeat open application test: Apply product to unaffected area e.gg forearm for 1-22 weeks
41
List 6 contraindications to patch testing
a. History of severe allergic reaction b. Generalized active dermatitis or angry back already c. Immunosuppressive treatment (up to 15 mg pred ok) d. Pregnancy/lactation e. Potent topical steroids on site of application within 2 days prior f. Recent sunburn -extensive g. Infection at site of intended application h. Unreliable patient/unwilling to comply with protocol
42
List 7 reasons for a false positive reading.
a. Irritant contact dermatitis b. Concentration too high c. Contamination d. Adjacent to a strong positive reaction e. Plaster reaction or allergy to finn chamber (aluminum) f. Angry back syndrome (often in patient with severe or widespread dermatitis) g. External manipulation or pressure artefact (e.g. scratching, bra strap) h. Uneven dispersion
43
List 6 reasons for a false negative reading.
a. Oral immunosuppression b. Recent sun exposure/sun burn c. Too weak dilution d. Patch falls off/poor placement e. Sweating or getting patched areas wet f. Reading results too early (e.g. no delayed reading for gold, drugs, steroids) g. Topical immunosuppression within days before test
44
What are 7 adverse reactions that can occur from patch testing that the patient must be counseled about.
a. Blistering reaction b. May trigger eczema flare c. Infection d. Pruritus e. Anaphylaxis f. Sensitization to new allergen g. Pigmentation to clothes (dye allergens) h. Hypopigmentation i. Scarring j. Angry back reaction
45
Name 5 formaldehyde-releasing preservatives
a. DMDM hydantoin b. Quaternium-15 c. Imidazolidinyl urea d. Diazolidinyl urea e. 2-bromo-2-nitropronane-1,3-diol (Bronopol) f. Tris(hydroxymethyl)nitromethane DQ BDI T
46
Name 6 potential cross-reacting substances to paraphenylene diamine
Sulfonamide antibiotics (TMP-SMX) Sulfonamide diuretics (HCTZ) Sulfonamide anti-diabetics (Glyburide) Benzacaine, procain, tetracaine PABA-para-aminobenzoic acid PTDA-paratoluene diamine Celecoxib Black rubber mix Disperse yellow, disperse orange
47
What are 3 co-sensitizers with nickel allergy?
Cobalt Chromate Palladium
48
what are 5 cross reactors with poison ivy
Cashew oil Mango rind Poison sumac Poison oak Japanese lacquer tree Gingko Balboa tree-fruit bulp Brazilian pepper tree Hawaiian Kahlil tree
49
What are 3 reasons why allergic contact dermatitis in leg ulcer patients is so prevalent?
Broken skin barrier Multiple topical preparations used Application under occlusion Chronic wounds
50
Top 5 allergens in patients with chronic leg ulcers
Fragrances: Balsam of peru, colophony Topical steroids HC, budesonide Anti-septics: chlorhexidine Antibiotics-bacitracin, neomycin Preservatives-MCI/MI, formaldehyde, paraben Vehicles-propylene glycol, lanolin
51
What are 4 groups of accelerators used in vulcanization of rubber that can result in allergic contact dermatitis
Thiurams Thioureas Carbamates Benzothiazoles
52
What are 3 antioxidants used in vulcanization that can cause allergic contact dermatitis to rubber?
Amines--> Diphenyl-p-phenylenediamine, IPPD Phenols--> Hydroquinone Dithioacids--> dibutyldithiocarbamate
53
What are 6 plant-derived food allergens that comprise the "latex-fruit syndrome" of cross reaction?
a. Bananas b. Avocado c. Bell pepper d. Potatoe e. Chestnut f. Kiwi g. Passionfruit h. Mango
54
What are the top 3 allergens causing non-occupational airborne allergic contact dermatitis?
a. Fragrances b. Sesquiterpene lactone c. Methylchloroisothiazolinone
55
What are the top 3 allergens causing occupational airborne allergic contact dermatitis?
Epoxy resins MCI/MI (cleaning) Formaldehyde Chrome Nickel
56
What are the top 3 allergens causing occupational airborne allergic contact dermatitis?
Epoxy resins MCI/MI (cleaning) Formaldehyde Chrome Nickel
57
12) What are 4 areas that you would examine to distinguish an airborne contact dermatitisfrom a photocontact/phototoxic dermatitis?
Retroauricular (Wilkinsons triangle) Eyelids Nasolabial fold submental
58
13) What are the 2 major species of poison ivy and their differences?
Toxicodendron radicans -eastern -clinbing vine Toxicodendron rydbergii -western -sprawling shrub
59
1) What does LASER stand for? What are the 3 C’s which characterize lasers?
a. Light amplification by stimulated emission of radiation b. Monochromicity (single or well-defined wavelength), coherence (light waves travel in phase), collimation (parallel beams)
60
2) What are the 3 different LASER media that exist and determine the laser wavelength?
- Gas (e.g argon, krypton, CO2) - Liquid (e.g. dye ) - Solid (e.g ruby, ND:YAG, alexandrite)
61
3) What are the 4 main safety concerns when using LASERS?
Cutaneous burns Fires Eye injury Biohazardous plume production from laser
62
4) List the 12 LASERS from Figure 9-1 in Alikhan, along with their wavelength (nm)
EAK PRAD NETEC Excimer: 308 Argon 488-514 KTP 532 PDL 585-600 Ruby 695 Alex 755 Diode 800 Nd:YAG 1064 Erbium: glass 1540 Thulium 1927 Erbium yağ 2940 CO2: 10600
63
5) List 3 chromophores that are frequent targets for LASERS.
Melanin Hb Water
64
List 2 vascular lasers
PDL Long pulse ND:Yag IPL Diode I love pulsed dye (IPL, Long pulse ND yag, PDL, Diode)
65
List 2 hair removal lasers
i. Diode, Nd:YAG (safest in richer skin tones), Alex, IPL DANI Diode, alex, nd , ipl
66
List 2 resurfacing lasers
Ablative: CO2, Er:YAG Non-ablative: PDL, diode, NdYaG, Erbium glass PEND)
67
Name 2 tattoo removing lasers
Q switch: Ruby Alexandrite ND-YAG * Again think melanin
68
List 4 commercially available botulinum toxins available. Provide both the generic name and the trade name for each.
a. Botox: Onabotulinumtoxin A b. Dysport: Abobotulinumtoxin A c. Xeomin: Incobotulinumtoxin A d. Nuceiva: prabobotulinumtoxi A e. Myobloc: Rimabotulinumtoxin B
69
List 5 broad categories of dermal fillers (based on material) and provide 1 example of each.
HA: Juvederm, restyle, parlance, beloetero Calcium hydroxyapatite: Radiesse Poly-L-Lactic acid: Scultpra Silicone: Silikone 1000 PMMA/bovine: Bellafill/Artefill Autologous fat Autologous fibroblasts
70
9) What type of dermal filler is each of the following (based on material) a. Zyderm: b. Restylane: c. Perlane: d. Belotero: e. Juvederm: f. Artefill: g. Sculptra: h. Radiesse:
a. Zyderm: Bovine collagen b. Restylane: HA c. Perlane: HA d. Belotero: HA e. Juvederm: HA f. Artefill: PMMA in Bovine collagen g. Sculptra: Poly-L-Lactic acid h. Radiesse: Calcium hydroxyapatite
71
List 5 complications that can occur with dermal fillers.
Blindness Necrosis/ulceration Ecchymoses Nodules Blue nodules Anaphylaxis
72
3 categories sclerotherapy agents and 2 examples of each
a. Hyperosmotic: Hypertonic saline (12-23%), Hypertonic saline 10% + dextrose 25% b. Chemical irritants: Glycerin, Polyiodide iodide c. Detergents: Sodium tetradecyl sulfate, Polidocanol
73
4 contraindications to sclerotherapy
a. Allergy to sclerosants b. DVT c. Advanced arterial occlusive disease d. Syptomatic PFO (for foaming sclerosants only)
74
What are 8 complications of sclerotherapy?
Urticaria Intraterial injection Ulceration/cutaneous necrosis Telangiectatic swelling Swelling Anaphylaxis Pain Ecchymoses PIH
75
What are the different categories of peels (based on depth)? Give 1 examples of each.
a. Superficial (papillary dermis) i. Salycyclic acid, glycolic acid, TCA 10-25%, Jessner’s b. Medium (upper reticular dermis) i. TCA 35-50% c. Deep (Deep reticular dermis) i. TCA >50%
76
15) What are components of Jessner’s solution?
LESR Lactic acid Ethanol Salicyclic acid Resorcinol
77
1) List the 4 motor nerve danger zones on the head and neck. Describe the following: i) Nerve affected; ii) Associated Adverse Events; iii) How to localize/landmark each danger zone.
a. Zygomatic arch/temple: Temporal nerve  brow ptosis (unilateral frontalis paralysis). Draw a line from 0.5 cm below the tragus diagonally to 1.5 cm above the lateral brow, third line from lateral orbital rim along the zygoma, forms a triangle b. Malar cheek: Zygomatic nerve inability to close eye, corneal desiccation. Mark highest point malar eminence, mandibular angle, oral commissure, forms a triangle. c. Mandible: Marginal mandibular nerve Inability to raise mouth to smile, drooling. 2-3 cm inferolateral to lateral oral commissure as traverses over mandible. d. Erb’s point: Spinal accessory nerve winged scapula, unable to abduct arm. 6.5 cm inferior to mastoid process in belly of SCM.
78
List 9 absorbable sutures (generic name and trade name).
-Fast absorbing gut -Plain gut - chromic gut - Fast absorbing polyglactin (Vicryl rapide) - Polyglactin 910 (Vicryl) - Polyglecaprone (Monocryl) -Polyglycolic acid (Dexon) -Polyglyconate -Polydioxanone (PDS 11)
79
List 5 non-absorbable sutures (generic name and trade name).
a. Silk b. Nylon (Ethilon) c. Polyprolene (Prolene) d. Polyester (Ethibond) e. Polybutester (Novafil)
80
Which absorbable suture has highest tissue reactivity? Which has the lowest?
Surgical gut > monocryl (polyglecaprone)
81
Which non-absorbable suture has highest tissue reactivity? Which has the lowest?
Silk > prolene
82
Which absorbable suture has initial tensile strength? Which has the lowest?
Monocryl (polyglecaprone) > plain surgical gut
83
Which non-absorbable suture has initial tensile strength? Which has the lowest?
Ethibond (of the non-metals)> silk
84
List 8 antiseptic used in dermatologic surgery. List 2-3 key points about each (bolded items in table 8-12).
a. Alcohol – fastest onset, broad spectrum, but inactive against spores and soiled hands, flammable b. Chlorhexidine - rapid onset, broad spectrum, but toxic to eyes and ears, inactive against spores, longest acting c. Iodine and iodophors- broad spectrum including bacterial spores, inactivated by blood and sputum, need to be dry to be effective, skin irritation d. Soap and water- best for hands, great for norwalk and c.diff e. Triclosan – binds enoyl-acyl carrier protein reductase, not overly effective f. Quaternary ammonium compounds (Benzalkonium) – slow, inactivated by organic compounds (e.g. gauze), used in eyedrops g. Chloroxylenol- slow, ineffective against pseudomonas h. Hexachlorphene – NO LONGER USED-neurotoxic, teratogenic
85
9) What is the difference between electrosurgery and electrocautery?
a. Electrosurgery: high frequency alternating current to conduct energy through cold tipped electrode, relies on poor conductance of human tissue to halt the current and convert electrical energy into thermal energy, which allows for hemostasis b. Electrocautery: direct application of heat to tissue through a hot tipped electrode generated by a direct current, there is no current flowing through the patient
86
Name 4 examples electrosurgery and 1 use for each, and whether biterminal or monoterminal
Mono: Electrofulguriation- zap off seb k Electrodescciation- ED&C Biterminal: Electrocoagulation -hemostasis Electrosection- surgery
87
What is mono vs. biterminal
Mono=no grounding terminal Biterminal= grounding terminal (grounding pad or biterminal forceps)
88
Safest method to use in ICD/pacemakrers?
Electrocautery If electrosurgery-biterminal
89
11) What are the 4 stages in graft uptake physiology (include timings).
Imbibition: 24-48 hours Inosculation-48-27, continues for 7-10 days Neovascularization- finished by day 7 Renervation- 2mo-years
90
Differentiate full thickness skin graft from split thickness skin graft on: Tissue match durability infection risk Nutritional requirments Adnexal structure function Wound contraction Sensation
FSTG better/good/higher in: -better tissue match, slightly better durability, better sensation, better adnexal function -higher nutritional requirements than STSG -less wound contraction Both low infection risk
91
List 7 surgical complications and the time frame when they usually occur.
Infection: 3-4 days Bleeding: first 24-48 hours Dehiscence: highest risk suture removal, first 1-2 weeks Poor wound healing/abnormal: variable Hematoma: 0-3 weeks ischemia/necrosis: flaps first 24-48 hours Nerve impairment-?immediate
92
List 7 surgical complications and the time frame when they usually occur.
Infection: 3-4 days Bleeding: first 24-48 hours Dehiscence: highest risk suture removal, first 1-2 weeks Poor wound healing/abnormal: variable Hematoma: 0-3 weeks ischemia/necrosis: flaps first 24-48 hours Nerve impairment-?immediate
93
List 6 broad categories of non-surgical modalities for scar improvement.
Massage Pressure therapy Topicals- e.g. silicone Injection-ILK Laser Radiofrequency
94
15) List 4 broad categories of surgical modalities used for scar improvement.
Dermabrasion Subcision Excision Re-orienting/lengthening
95
What is the scar tensile strength at 1 week, 3 weeks, 3 mo, 1 yr
5% at 1 week 20% at 3 weeks 40% at 3 months 80% 1 yr
96
List 5 streptococcal skin infections
Eryispelas Cellulitis* Non bullous impetigo Blistering dactylitis Necrotizing fascitis Ecthyma Perianal strep Scarlet fever Toxic shock syndrome
97
List 5 staphylococcal infections
Impetigo-bullous/non-bullous Cellulitis Abscess/furuncle/carbuncle Folliculitis Staph scalded skin syndrome Toxic shock syndrome Pyomyositis Botryomycosis
98
List 5 pseudomonal skin infections.
Green nail syndrome Otitis externa, malignant otitis externa Psuedomonal folliculitis Psuedomonal pyoderma Psuedomonal hot foot Ecthyma gangrenosum
99
List 10 different forms of cutaneous tuberculosis (broad categories: exogenous, endogenous, and tuberculid)
Exogenous: 1. Tuberculous chancre 2. Tuberculosis verruca cutis (high) Endogenous: 3. Lupus vulgaris (high) 4. Scrofuloderma 5. Miliary TB 6. Tuberculous gumma 7. Oroficial TB Tuberculid: All high immunity 8. Lichen scrofulosum 9. Papulonecrotic tuberculids 10. Erythema induratum of basin
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5) List the types of leprosy according to the Ridley and Jopling classification system.
Tuberculoid (th1/high cell immunity) Bordeline tuberculoid Borderline borderline Borderline lepormatous Lepromatous (Th2, low cell)
101
List 6 cutaneous nerves that run superficially and should be palpated to assess for thickening in leprosy patients:
Medial radial ulnar Greater auricular CN 5 CN 7 Posterior tibial Common perineal
102
List 6 dermatophytes that demonstrate endothrix growth during hair infection
Ringo Gave Yono Two Squeaky Violins Trichophyton rubrum, T.Gourvilli, T.Yaounde, T.Tonsurans, T.Soudanense, T.Violaceum
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8) List 5 dermatophytes (ectothrix) that fluoresce on Woods lamp examination due to the presence of what substance:
Pteridine Cats And Dogs Fight and Growl Sometimes M. canus M. audouinii M. Distortum M. Ferrugenium M. Gypseu, T. Schloenni
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What causes favus
T Schloenni *May fluoresce dull gray green
105
Most common causes tinea wapitis: -us -worldwide -favus -kerion
Trichophyton tonsurans (#1 cause in United States) Microsporum canis (#1 cause worldwide; more inflammatory) T. violaceum (East Africa) Endothrix (black dot; arthroconidia within hair shaft): Ringo Ectothrix (arthrospores around hair shaft) 1. Fluorescent (green via Wood lamp – pteridine): “Cats And Dogs" 2. Nonfluorescent: MMR NGV T. mentagrophytes, T. rubrum, M. nanum, T. megninii, T. gypseum, and T. verrucosum 3. Favus – T. schoenleinii mainly 4. Kerion – M. canis, T. verrucosum, T. mentagrophytes, and T. tonsurans
106
What are 4 clinical variants or presentations of tinea capitis?
Non inflam: Grey Patch (ecto) Black dot (endo) Diffuse scale Inflammatory: Kertion Favus Diffuse pustular
107
What are 4 clinical presentations of tinea pedis?
Moccasin Interidigital Acute ulcerative Acute vesiculobullous
108
List 4 clinical types of onychomycosis:
DLSO Proxima white subungual Superficial white Total dystrophic Endonyx
109
What are the vectors for leishmaniasis
Sand flies Old world-Phlebotomus New world-Lutzomyia
110
List 3 species that cause old world leishmaniasis
Major Tropical Infants in Ethiopia a. Leishmania major, L. Tropica, L. infantum, aethiopica
111
List 3 species that cause new world leishmaniasis
a. L. mexicana, L. brasiliensis, L. amazonensis BAM
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15) What are 5 broad clinical variants of leishmaniasis?
Cutaneous-new and old Diffuse cutaneous Mucocutaneous Visceral (Kala Azar) Post Kala Azar
113
What are 5 risk factors for cutaneous larva migrans (CLM)?
Male, young/children, barefoot/bum, travelers to tropical climates (jamiaca, Dominican, Thailand), swimmers, broken skin, wet sand, beach with many cats/dogs
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What is one major difference between new and old world leish? TOC visceral and cutaneous leash
Not confirmed a. Old world cutaneous--> typically presents with single ulcer verrucous w/ sporotrichoid spread, new world usually more varied: ulcerations (Chiclero ulcer = ear lesion in workers who harvest chicle gum in forest), impetigo-like, lichenoid, sarcoid-like, nodular, vegetating, and miliary b. Mucocutaneous is almost always new world c. Most cases of old world resolve in 15 months. 75% of L. Mexican new world resolve ) Mucocutaneous (new world) does NOT self resolve ((L. braziliensis and L. panamensis) does NOT self-resolve and requires treatment to prevent progressive destruction) Visceral more old world Cutaneous/mucocutaneous: pentavalent antimony Visceral leishmaniasis: Amphotericin B (ToC)
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18) List 2 organisms responsive for CLM.
Ancylostoma brasiliensis Ancylsotoma caninum
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19) List 5 treatment options for CLM.
Albendazole Ivermectin Thiabendazole-topical and oral Liquid nitrogen
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20) What is the classic cutaneous eruption seen in strongyloides? What is the treatment of choice for strongyloides?
Thumbprint purpura Ivermectin
118
List 5 genodermatoses that can be associated with CALMS
a. NF I, NF II, Mosaic NF I (NF 5) b. Legius syndrome c. McCune Albright syndrome d. Bloom syndrome e. Noonan syndrome (Allelic w/ LEOPARD syndrome/Noonan with multiple lentigines) f. Tuberous sclerosis g. Fanconi h. Ataxia telangiectasia i. MENI j. Constitutional mismatch repair k. Ring chromosome syndrome l. Cowdens m. Proteus syndrome n. Watson syndrome
119
List 5 syndromes associated with multiple lentigines.
a. Noonan syndrome with multiple lentigines (Formerly known as LEOPARD) b. Carney complex (LAMB/NAME) i. Lentigines, atrial myxoma, blue nevus ii. Nevi, atrial myxoma, myxoid neurofibromas, Ephelids c. Laughier-Hunziker (Oral, genital, melanonychia, r/o peutz Jegher) d. Peutz Jegher (intra-oral) e. Cowdens (penile-PTEN) f. Bannayan-Riley-Ruvalcaba (penile-PTEN) g. Xeroderma pigmentosum
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What does LEOPARD stand for
L stands for (L)entigines (E)lectrocardiographic conduction defects (abnormalities of the electrical activity and the coordination of proper contractions of the heart); (0)cular hypertelorism (widely-spaced eyes); (P)ulmonary stenosis (obstruction of the normal outflow of blood from the right ventricle of the heart); (A)bnormalities of the genitals; (R)etarded growth resulting in short stature; (D)eafness or hearing loss due to malfunction of the inner ear (sensorineural deafness).
121
List 5 variants of dermal melanocytosis
a. Congenital dermal melanocytosis (Mongolian spots) b. Nevus of ota c. Nevus of ito d. Hori’s nevus (acquired) e. Sun’s nevus (acquired, unilateral Hori’s)
122
Which mutations are often associated with Blue Nevus
GNAQ GNA11
123
Which gene is associated with inherited form of melanoma? Which proteins does it code for?
CDKN2A--> FAMM Familial atypical multiple melanoma P14-ARF and P16--> modulate cell cycle progression through P53 and Rb pathways, respectively
124
Name 8 mutations seen in melanomas
BRAF --> V600E MC (sup spreading) NRAS C-KIT (mucosal and acral) CCND1/CDK4 TERT GNAQ/GNA11: uveal melanoma, blue nevi, and nevus of Ota BAP-1: Cutaneous melanoma, uveal melanoma, malignant cellular blue nevus, epithelia spitzoid nevi, MESOTHELIOMA, and renal cell carcinoma may be seen in a syndrome which is associated with which mutation?
125
List 5 variants melanoma
a. Nodular b. Superficial spreading c. Lentigo maligna melanoma d. Acral lentiginous melanoma i. Uveal melanoma ii. Mucosal melanoma iii. Desmoplastic melanoma iv. Spitzoid melanoma v. Malignant blue nevus
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Acral and mucosal melanomas are associate with which mutations
C-KIT
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Superficial spreading melanomas are associated with which mutation
BRAF-V600E
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10) Uveal melanomas and blue nevi are associated with which mutation
GNAQ/GNA11
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11) Cutaneous melanoma, uveal melanoma, malignant cellular blue nevus, MESOTHELIOMA, and renal cell carcinoma may be seen in a syndrome which is associated with which mutation?
BAP-1
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12) According to AJCC 8, what do the following correspond to: Name the T stages for melanoma
T1a- <0.8 mm no ulceration (1A) T1b- <0.8 mm w/ ulceration, or 0.8-1.0mm (1B) T2a- >1-2.0 m w/out ulceration (1B) T2b- >1-2.0 mm w/ ulceration (2A) T3a- >2-4.0 mm w/out ulceration T3b- >2-4.0 w/ ulceration T4a- > 4 mm w/o ulceration T4b- > 4 mm w/ ulceration
131
Name the clinical staging for melanoma according to AJCC 8 edition
IA: T1a (anything <0.8 mm w/out ulcer) IB: T1b, T2a (anything < 1mm w/ ulceration, 0.8-2 w/out ulceration 2A: T2b, T3a 2B: T3b, T4a 2C: T4b 3: Any T N>1 M0 4: Any T any N M >1
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Which two gene mutation have been associated with seborrheic keratoses? List 7 clinical variants of SKs. List 6 histological variants of SKs.
PIK3CA FGFR3 Variants: -inverted follicular keratosis -lichenoid keratosis -stucco keratosis -acrokeratosis verruciformis of hopf -dermatosis papulosa nigra -clear cell acanthoma -large cell acanthoma 6 path variants: "CHRAIM" -hyperkeratotic -acanthotic -clonal -irritated -reticulate - melanoacanthoma
133
List 10 risk factors for invasive SCC
Ia. Immunosuppressed, CLL b. Older age c. Male d. Fair skin e. Genetic conditions: e.g. XP f. UV- chronic>intermittent g. HPV h. Radiation i. Chronic non healing wounds-Marjolin ulcer j. Inflammatory dermatoses such as DLE, erosive oral LP, chronic LSA k. Medications: BRAF inhibitors, voriconazole
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List 6 genetic syndromes associated with SCC.
a. Photosensitive: i. Oculocutaneous albinism ii. Xeroderma pigmentosum iii. Rothmund thompson b. The D’s: i. EDV ii. Dystrophic EBA iii. Dyskeratosis congenita iv. KID syndrome-keratitis, ichthyosis, deafness c. Misc: i. Porokeratosis-linear ii. Werner syndrome iii. Chronic mucocutaneous candidiasis
135
What is verrucous carcinoma? Which HPV subtype is it associated with? What are the 3 clinical variants and where on the body does each variant present?
Low grade, locally destructive SCC HPV 6/11 Clinical variants: Buschke lowenstein Tumor - perianal Epithelium cuniculatum - foot Oral florid papillomatosis - oral cavity Papillomatosis cutis carcinoides of Gottron
136
List 6 clinical variants of keratoacanthoma. What are 2 KA specific syndromes?
Variants: Solitary Multiple Intraoral Subungual Giant Keratoacanthoma centrifigum marginatum KA syndromes: Gryzbowski Ferguson-Smith
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Describe the two KA syndromes- acquired vs. genetic, onset, appearance
Ferguson-Smith-AD, rapid onset multiple large KAs, resole spont. 3rd decade Gryzbowski: Sporadic, thousands milia-like KAs, later in life, can have airway involvement, scarring, ectropion
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What is the most common mutation identified in BCCs. List 8 variants of BCCs. List 6 genetic syndromes associated with BCCs.
a. PTCH most common mutation b. Variants i. Superficial ii. Nodular iii. Morpheaform iv. Fibroepithelioma of Pinkus v. Micronodular vi. Basosquamous vii. Infundibulocystic viii. Pigmented Genetic syndromes i. Basal cell nevus syndrome, Brooke Spiegler ii. OCA, XP, Bloom syndrome, Rothmund Thomson, Werner iii. Bazex Dupre Christol, Rombo syndrome, Schopf Shulz Passarge
139
Which syndrome is associated with multiple epidermoid cysts. What are other features of this syndrome?
a. Gardner’s syndrome-APC (adenomatous polyposis coli) i. Skin: Epidermoid cysts w/ pilomatricoma features, lipomas, fibromas (skin, subcutaneous, mesentery, retroperitoneal), osteomas ii. GI manifestations: Desmoid tumors, premalignant polyposis iii. Eyes: CHRPE Cancer: colon duodenal , brain liver adrenals thyroid iv. Others: Osteomas (subcutanesous), odontomas, supernumary teeth, sarcoma, papillary thyroid cancer (women), adrenal adenomas, brain tumors (gliblastomas and medulloblastoma in Turcot syndrome), pancreatic carcinomas
140
Who most frequently gets dermoid cysts and where are they most commonly located?
a. Along embryonic fusion lines, most common lateral eyebrow b. Infants
141
Which syndrome is steatocystoma multiplex associated with? What are the gene mutations and other features of this syndrome?
a. Pacyhonychia congenita Type II b. Gene mutations: KRT17>>KRT6B c. *In general: PPK, painful heels, thickened nails d. Features: i. Natal teeth ii. Oral leukokeratosis iii. Steatocystoma or other cysts iv. Palmar plantar keratoderma-milder than type I
142
What are the 3 main findings in Brooke Spiegler
Trichoepithelioma Spiradenoma Cylindroma Trichoblastoma
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Which syndrome is cylindroma associated with? What is the gene mutation? What are the other features of this syndrome?
Brooke Spiegler Gene mutation: CYLD Features i. Cylindroma (papules/nodules scalp) ii. Trichoepithelioma (facial papules) iii. spiradenoma (painful nodules head neck) iv. Trichoblastomas,
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What are 5 cancers that can occur in Brooke Spiegler
BCC Spiradenocarcinoma Cylindrocarcinoma Salivary and parotid gland tumors
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What syndrome is associated with multiple fibrofolliculomas/trichodiscomas? What is the gene mutation? List all cutaneous and 4 non-cutaneous features.
Birt-Hogg-Dube Gene: FLCN (folliculin) Cutaneous: i. Fibrofolliculoma ii. Trichodiscoma iii. Achrocordons iv. Angiofibromas Non cutaneous features: i. RCC ii. Spont. Pneumothorax iii. Pulmonary cysts iv. Medullary thyroid cancer v. Parotid gland onycocytomas
146
Which secondary adnexal neoplasms can arise in a nevus sebaceous? Which is the most common?
2 S's, 2 T's and a B a. Trichoblastoma -most common b. Trichilemmoma c. Syringocystadenoma papilliferum d. Sebaceoma e. BCC f. Others: i. Eccrine poroma ii. Tubular apocrine adenoma
147
List 2 syndromes with multiple trichoepitheliomas.
Brooke Spiegler Rombo
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Which gene/protein mutation is associated with pilomatricomas? List 4 syndromes associated with pilomatricomas.
CTNNB1 Gene--> B catenin Myotonic dystrophy Rubinstein Taybes Gardner-pilomatricoma features in epidermoid cysts Turners
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What syndrome are multiple trichilemmomas associated with What gene is mutated in this syndrome? What are other features of this syndrome? List malignancies that are associated with this syndrome.
a. Cowden’s b. PTEN mutation i. LA HAS TOPS BET 1) Lipomas, angiolipomas, hemangiomas, acral keratoses, skin tags, trichelommomas, oral papillomas (cobble stoning to lips/gingival/oral mucosa), sclerotic fibromas Malignancy: Breast, thyroid, endometrial Others: Fibrocystic breasts, thyroid goiter, GI tract hamartomous polyps (low risk), ovarian cysts, uterine leiomyomas, menstrual irregularities, bony cysts, kyphoscoliosis, craniomegaly, large hands/feet, adenoid facies, angiod streaks, myopia
150
What is the inheritance pattern for Muire-Torre syndrome (MTS)? Which genes are associated with this? What is the clinical presentation for MTS? Which malignancies are patients with MTS at an increased risk for?
a. Inheritance pattern: AD b. Phenotypic variant of Lynch c. Genes: MLH1, MSH2 (90%), MSH6, PMS2 Sebaceous adenoma, sebaceous carcinoma, sebaceoma *Esp non facial, multiple KAs often w/ sebaceous differentiation (strong) At risk for: Colon cancer #1, GU #2 (bladder, kidney, ureter etc.), gastric, ovarian, endometrial
151
17) Differential for painful skin tumors
BLEND AN EGG Blue rubber bleb nevus Leiomyoma Eccrine spiradenoma Neuroma Dermatofibroma Angiolipoma, angioleiomyoma Neurilemmoma Endometrioma Gloms tumor Granular cell tumor
152
18) Which syndrome is associated with multiple cutaneous leiomyomas? What is the gene mutation? What are the other clinical features?
Hereditary Leiomyomatosis and RCC syndrome/Reed syndrome Fumarate hydratase RCC Leiomyomas-cutaneous and uterine
153
List 5 path variants of dermatofibroma.
Cellular DF Hemosiderotic Aneurysmal Monster cells (DF with monster cells) Xanthomatous
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b. List 4 clinical (superficial) variants of fibromatosis.
Palmar Plantar Peyronies Knuckle pads
155
List 3 clinical differences between and infantile hemangioma and vascular malformation.
a. Growth: IH rapidly proliferate for 4-6 mo, VM do not b. Resolution: IH can spont. Resolve, VM either persist or resolve c. Onset: IH not present at birth, VM present at birth d. Appearance: IH are usually bright red but can be purple-blue if deeper; VM have variable clinical appearances but tend to persist and become more verrucous over time
156
Compare the epidemiology of infantile hemangiomas and vascular malformation.
IH: 4-5% of infants, more in Caucasians, F>M Vascular malformation: F=M, most common are CMs, least common AV, low flow more common
157
What are the three phases in the natural history of an infantile hemangioma
Proliferating, plateau, involution
158
List 5 risk factors for infantile hemangiomas.
female sex multiple gestations-twins placental anomalies premature advanced maternal age low birth weight
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5) What are 4 potential complications of infantile hemangiomas?
a. Ulceration b. Disfigurement – e.g. nasal tip, breast asymmetry, fibrofatty residual c. Interference with function from location- e.g. periocular and vision loss d. Extracutaneous involvement-e.g. internal hemangiomas, PHACES, beard hemangioma and laryngeal hemangiomatosis Also death from airway involvement or high output cardiac failure
160
List 3 immunohistochemical markers that can be used to differentiate infantile hemangioma from vascular malformation.
a. GLUT-1 b. Lewis Y-Antigen c. Merosin d. Wilm tumor protein (WT-1) e. FcγRII f. *All positive in IH, negative in vascular malformation
161
What are the 4 segmental hemangioma patterns?
Frontotemporal Maxillary prominence Mandibular prominence Frontonasal
162
What does PHACES stand for
a. P: Posterior fossa malformations (e.g., Dandy-Walker and cerebellar hypoplasia) b. H: Hemangioma, segmental c. A: Arterial anomalies (internal carotid arteries and cerebral arteries) d. C: Cardiac anomalies (coarctation of aorta, ventral and atrial septal defects, and patent e. ductus arteriosus) f. E: Eye anomalies (microphthalmos, optic atrophy, cataracts, strabismus, and exophthalmos) g. S: Sternal cleft or supraumbilical raphe
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What is LUMBAR syndrome
Lower body/lumbosacral/lipoma /hemangioma Ulceration/urogenital anomalies Myelopathy (spinal dyspraphism) Bony deformities (hip dysplasia, scoliosis) Anorectal anomalies Renal anomalies (hypo plastic single kidneys, bladder/ureter anomalies)
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What does SACRAL stand for?
a. Spinal dysraphism b. Anogenital anomalies c. Cutaneous anomalies d. Renal/urologic anomalies e. Angioma in Lumbosacral area
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What does PELVIS stand for?
- P: Perineal haemangioma - E: External genitalia malformations - L: Lipomyelomeningocele - V: Vesicorenal abnormalities - I: Imperforate anus - S: Skin tag
166
List 3 clinical differences between infantile hemangiomas and RICH/NICH.
o Onset: IH develops in the first few months of life; RICH/NICH is apparent at birth o Growth: IH undergoes rapid growth for 4-6 months followed by slow involution over years; RICH/NICH are fully developed at birth o Resolution: RICH will involute over 1 year, IH will involute over several years, NICH will not involute
167
List 2 histopath differences in RICH/NICH vs. IH
IH: GLUT-1, WT-1, Lewis-Y-antigen POSITIVE (vs. RICH/NICH negative) 1. striking lobularity with densely fibrotic stroma 2. stromal hemosiderin deposits, 3. focal thrombosis and sclerosis of capillary lobules, 4. fewer mast cells 5. coexistence of proliferating vasculature with multiple thin-walled vessels
168
What are 4 reasons to consider systemic therapy for infantile hemangioma?
a. Threaten vital function- vision, airway b. Threatent life e.g. High output heart failure, airway involvement c. Potential for disfigurement: columella, nasal tip, ear, rapidly growing lesion on face d. Ulceration-severe or recalcitrant
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What are the 5 major goals of management of infantile hemangiomas?
a. Preventing or reversing life or function- threatening complications b. Preventing potential disfigurement c. Treating ulcerations d. Minimizing psychosocial impairment to patient and families e. Avoiding overly aggressive procedures that could cause scarring
170
What are 6 extractable antigens
SSA-ro SSB-la Smith SCL-70 RNP (U1RNP)--> high titers → MCTD; lower titles → SLE J0-1
171
What are 3 non ENA targets
Centromere dsDNA Histone
172
List 3 antibody targets that are highly specific for SLE. What are they associated with?
dsDNA: lupus nephritis and activity, lupus disease activity, sun-protected non lesional skin lupus band Smith- not much, more prevalent in Asian/African American patients rRNP (ribosomal)-->neuropsychiatric *NB 1. rRNP with lupus/neuropscyh lupus 2. RNA polymerase I/III with systemic sclerosis 3. U1RNP with MCTD
173
List 5 dermatomyositis associated antibodies, not including anti-synthetase syndrome. What are they associated with?
4) List 5 dermatomyositis associated antibodies, not including anti-synthetase syndrome. What are they associated with? a. Mi-2: Classic DM findings, good tx response, no malig or ILD risk Tiff-1-gamma (P155/P140): A/hypomyopathic, ++ malignancy, GI w/ severe dysphagia, severe skin (ovoid patch, psoriasiform lesions, palmar hyperkeratosis, atrophic hypopigmented patches w/ telangiectasias), No RP/ILD/arthritis i. JDM: no malignancy, treatment refractory, lipodystrophy MDA-5: Amyopathic, rapidly progressive ILD specific skin (cutaneous vasculopathy with oral/skin ulcers, tender palmar papules, mechanics hands, panniculitis) - JDM ulcers, ILD, arthritis, mild muscle (i.e. similar) NXP2 (P140, MJ): Malignancy in adults, calcinosis, peripheral edema, -JDM: Severe calcinosis, severe muscle involvement, vasculopathy w/ gi bleeds SRP: fulminant PM/DM, cardiac involvement, poor prognosis SAE: Severe cutaneous dz, progressive muscle disease w/ dysphagia, fever/weight loss, erythroderma if given HCQ g. Anti-synthetase: Jo-1, PL7, PL-12, OJ, EJ i. Others include ARS, KS, HA, ZA
174
Which antibody is most specific for CREST? What is it associated with?
a. Anti-centromere--> Calcinosis, raynauds, esophageal dysmotiliy, sclerodactyly, telangiectasias b. Associated with pulmonary HTN
175
Which antibody is most strongly associated with primary systemic sclerosis? What is it associated with?
a. SCL-70/Topoisomerase b. Associated with ILD/pulm fibrosis *RNA polymerase III is the third ab and related to renal crisis and severe skin
176
7) Which two antibodies are associated with linear morphea?
ssDNA (correlated w/ disease activity) Anti-histone (lin and generalized, correlated w/ dz activity)
177
Which two antibodies can be detected in patients in RA? What are they associated with?
RF: -high levels associated with severe erosive RA, vasculitis and neuropathy, can also be seen in mixed cryoglobulinemia -Low level RF non specific CCP: severe RA, development of RA
178
List 3 autoantibodies associated with Sjogren's syndrome. What are they associated with?
SSA (neonatal LE and SCLE) SSB alpha fodrin (actin binding product)
179
Which antibody is detected in neonatal lupus?
RO/ssA
180
11) Which antibody is associated with mixed connective tissue disease?
U1RNP
181
List the components of the newest (EULAR/ACR) criteria for SLE (more recent than the SLICC criteria).
*Need 10 points Must have + ANA for entry criteria Constitutional domain: Fever Cutaneous: ACLE, SCLE/DLE, non scarring alopecia, oral ulcers Arthritis: synovitis or tenderness in 2+ jonts Renal: Lupus nephritis, proteinuria Serositis: Pleural or pericardial effusion, acute pericarditis Neurologic: Delirium, psychosis, seizure Labs: Leukopenia, thrombocytopenia, hemolytic anemia Immunologic i. dsDNA or smith ii. APLS: ACA or LAC or B2GP iii. Low C3 and/or C4
182
List the classification for dermatomyositis.
Adult -Classic -DM overlap -Amyo -Hypomyo -Malignancy assoc. Juvenile -classic -hypomyo -amyopathic
183
List 5 variants of morphea
Localized: plaque, superficial, atrophoderma passini and piereni, , guttate, nodular, bullous Linear: en coup de sabre, Perry-romberg, trunk-limb, atrophoderma of moulin Generalized Pansclerotic Mixed
184
Skin findings in RA
a. Rheumatoid nodules b. Rheumatoid vasculitis i. Small, medium, EED, Bywaters lesions c. Livedo reticularis and livedoid vasculopathy d. Neutrophilic dermatoses i. PG ii. Sweet’s iii. Rheumatoid neutrophilic dermatitis iv. Palisading neutrophilic granulomatous dermatitis v. Interstitial granulomatous dermatitis vi. Superficial ulcerating rheumatoid necrobiosis MTX induced papular eruption
185
List 5 entities on the ddx for systemic sclerosis.
GVHD Nephrogenic systemic fibrosis Scleredema, scleromyxedema, pretibial myxedema Morphea Eosinophilic fasciitis Others: Mucinoses Localized: radiation induced, injection site (vitamin K, bleomycin) Generalized: toxic oil syndrome, polyvinyl chloride, belomycine, taxanes, eosinophilic-myalgia (L tryoptophan) Paraneoplastic: POEMS, carcinoma en cuirasse, amyloidosis, carcinoid Metabolic: PCT, diabetic cheiropathy (diabetic stiff hand syndrome)
186
What are the different types of scleredema and their associations?
Post infectious- Type I Monoclonal gammopathy related – Type II Diabetic related -Type III
187
3) What is POEMS syndrome?
Polyneuropathic organomegaly endocrinopathy monoclonal gammopathy Skin changes-melanoderma/hyperpigmentation, hemangioma, hypertrichosis
188
List 4 major perforating disorders and 1 association with each.
a. Elastosis perforans serpiginosa (EPS): i. MAD PORES 1. Marfans 2. Acrogeria 3. Downs 4. Penicillamine, PXE 5. OI 6. Rothmund Thompson 7. EDS 8. Scleroderma b. Acquired perforating dermatosis (APD): Diabetes, renal failure c. Reactive perforating collagenosis (RPC); Childhood onset, minor trauma d. Perforating calcific elastosis (PCE): Obese, hypertensive, multiparous women
189
What are 4 broad categories of cutaneous crohn’s disease?
a. Specific lesions: i. metastatic chrons, contiguous perianal or oral chrons ii. Oral: cobblestoning of the buccal mucosa, tiny gingival nodules, gingival hyperplasia, aphthae-like ulcers, linear, knife-like ulcerations, angular cheilitis and ulceration, cheilitis granulomatosa, diffuse oral swelling, or indurated fissuring of the lower lip b. Non-specific lesions/reactive i. erythema nodosum, PG (UC>chrons), pyostomatitis vegetans (UC>chrons), erythema multiforme, finger clubbing, cutaneous small vessel vasculitis, epidermolysis bullosa acquisita, vitiligo, palmar erythema, a pustular response to trauma (pathergy), c. Nutritional skin changes e.g. acrodermatitis enteropathica d. Treatment related skin change
190
What are the 5 clinical findings seen in Lofgren’s syndrome?
EN Fever hilar LAD migrating polyarthritis uveitis
191
7) What are the clinical findings seen in Heerfordt’s syndrome?
Uveitis Fever parotid gland enlargement CN palsies
192
8) What are the clinical findings seen in Mikulicz’s syndrome?
Salivary gland (parotid, submandibular) and lacrimal gland swelling
193
What are 5 examples of palisaded granulomas? (Figure 3-64)
a. GA (central mucin) b. RA nodule (Central fibrin) c. Necrobiotic xanthogranuloma (degenerative collagen) d. Necrobiosis lipoidica diabeticorum e. Annular elastolytic giant cell granuloma f. Uric acid crystals gout
194
List 6 different types of cutaneous xanthomas.
Eruptive Tendinous Tuberous Plane Xanthelasma-plane xanthoma on eyelids verruciform xanthoma
195
11) List 6 different types of physical urticarias.
a. Dermatographism b. Aquagenic c. Cholinergic d. Solar e. Vibratory f. Delayed pressure g. Cold-induced
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12) What is the clinical criteria for Sweet’s syndrome?
c. Major: i. Skin: abrupt onset painful cutaneous lesions ii. Path: consistent with sweets d. Minor i. Leukocytosis ii. Response to steroids iii. Associated Dz: infection, vaccinations/drugs, inflammatory condition (IBD, AI-CTD, sarcoid, bechets), malignancy, pregnancy 1. Drug induced: GO SHLAM a. G-CSF/GM-CSG b. OCP c. Septra d. Hydralazine e. Lasix f. ATRA g. Minocycline iv. Fever or constitutional symptoms
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13) What is the clinical criteria for pyoderma gangrenosum?
a. 2 Major + 2 Minor b. Major: i. Skin: Rapidly progressive painful cutaneous ulcer (50% growth in 1 mo), undermined, irregular violaceus border ii. Causes: Other causes excluded c. Minor i. Pathergy- Hx suggestive pathergy (minor trauma) or cribiform base scarring ii. Associated dz (IBD, IgA gammopathy, arthritis, malignancy) iii. Response to treatment (rapid response to steroids) iv. Path findings
198
What are 4 variants of pyoderma gangrenosum?
Bullous Ulcerative Pustular Superficial granulomatous/ vegetans Pyostomatitis vegetans/pyoderma also mentioned Peristomal
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What is the clinical criteria for Behcet’s disease?
a. Major (1/1): Recurrent apthosis 3+ episodes for year b. Minor (2/4) i. Pathergy ii. Eyes-anterior or posterior uveitis iii. Genital ulcers iv. Skin findings -EN, folliculitis
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16) What do the following acronyms stand for: i) PAPA; ii) PASH; iii) PAPASH; iv) PASS; v) SAPHO
a. PAPA: Pyogenic arthritis, Pyoderma gangrenosum and Acne b. PASH: Pyoderma gangrenosum, Acne and HS c. PAPASH: Pyoderma gangrenosum, pyogenic arthritis, Acne, and HS d. PASS: PG, Acne, HS, Ankylosing spondylitis e. SAPHO: Synoviits, acne, pustulosis, hyperostosis and osteitis
201
17) What are 3 different types of miliaria?
Cyrstalina-corneum Rubra-mid epidermis Profunda - DEJ
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18) List 5 causes of drug-induced acne:
18) List 5 causes of drug-induced acne: a. Prednisone b. Anabolic steroids c. Androgens-Testosterone d. JAK inhibitors e. Halogens f. Lithium g. Isoniazid h. Phenytoin i. EGFR inhibitors, MEK inhibitors, Mtor inhibitors j. IUD k. OCP l. Vitamin B2, B6,B12, biotin SHIELD yourself from Vitamin T i. “Steroids (anabolic, corticosteroids), Halogens, Isoniazid, EGFR inhibitors, Lithium, Dilantin (phenytoin), Vitamin B2, B6, B12, Testosterone and babies (OCP, IUD)
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19) How is acne fulminans different from acne conglobate?
a. Systemic symptoms in fulminans-fevers, arthralgia, myalgias, malaise, osteolytic bone lesions
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20) List 10 acne variants.
a. Acne vulgaris b. Acne conglobata c. Acne fulminans d. Acne excoriee e. Neonatal acne f. Infantile acne g. Acne cosmetica h. Pomaid acne i. Chloracne j. Radiation acne
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Name 5 GA variants
a. Generalized b. Annular c. Patch d. Perforating e. Subcutaneous/Deep GAPPS
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Ddx for eosinophilic spongiosis
BAD CHIP PIGS BP AD Drug Contact derm herpes gestationis/pempigoid IP PV PF Grovers scabies infestation
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2. What clinical sign is associated with papuloerythroderma of ofuji? What histological finding is characteristically seen with Well’s syndrome?
Deck chair sign Flame figures
208
What is the criteria for hypereosinophilic syndrome and what are the 2 major subtypes
peripheral eosinophils >1500/mm3 x 6 months or < 6 mo w/ end organ damage No evidence underlying cause (parasite, allergy, drug etc.) End organ damage i. Myeloproliferative: MC FIP1L1A-PDGFRA translocation, tyrosine kinase, can tx w/ imatinib, , elevated tryptase, b12, constitutional symptoms, endomyocardial fibrosis, Hepatosplenomegaly, bad prognosis ii. Lymphocytic: clonal proliferation that produced Th2 Il5, benign course but cardiac complications can occur still and increase risk T cell lymphoma,
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What is the # 1 cause death HES
CHF
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What are 5 common causes of flushing?
MR FAB D Menopause Rosacea Fever Alcohol Benign cutaneous flushing-emotion, hot, spicy foods, exercise Drugs: niacin, alitretinoin, ACEi, BB, CCB, doxorubicin, opioids, vanco, eicosanoids, rifampicin, steroids, gold, sildenafil
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What are 4 serious causes of flushing
A spicy red pineapple made me cry violently Anaphylaxis Scombroid poisoning RCC Pheo medullary thyroid cancer mastocytosis carcinoid ViPoma
212
What are 3 variants of eosinophilic folliculitis?
Eosinophilic pustular folliculitis of ofujii Neonatal eosinophilic pustular folliculitis HIV-associated
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What is the follicular occlusion tetrad?
HS Acne conglobata Pilnodal sinus Dissecting cellulitis
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What are the clinical findings seen in Hurley stage 1, 2 and 3 HS
a. Stage 1: Solitary or multiple isolated abscesses, with no scarring or sinus tracts b. Stage 2: Recurrent abscesses, with sinus tract or scarring c. Stage 3: multiple interconnected sinus tracts and abscesses throughout an affected region; more extensive scarring (entire area usually)
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What are the 3 diagnostic criteria for HS
1. typical lesions of deep-seated painful nodules (known as “blind boils” without a purulent point) in early lesions and abscesses, sinuses, bridged scars, and “tombstone” open comedones (pseudocomedones) in secondary lesions. Lesions occurring in at least 1 typical body location such as the axillae, groin, perineal and perianal region, buttocks, and inframammary and intermammary folds. Chronic nature of disease, relapses, and recurrences.
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9. List 5 medications that can cause DRESS
a. Allopurinol b. Antibiotics (septra, minocycline) c. Anti-inflammatory- NSAIDS d. Anticonvulsants (carbamazepine, phenobarbital, phenytoin, VPA, lamotrigine) e. AZA, abacavir and nevirapine f. Dapsone
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10. List 5 medications that can cause AGEP.
LiT BECA Lamisil/terbinafine Tetracycline Beta lactam Erythromycin/macrolides CCB-diltiazem Anti-malarial
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11. List 5 medications that can cause pseudoporphyria:
LATINO Lasix Amiodarone Tetracycline Isotretinoin Naproxen OCP
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12. List 5 medications that can cause a morbilliform drug eruption.
a. Penicillins b. Cephalosporin c. Anticonvulsants d. Allopurinol e. TMP-SMX
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13. List 5 medications that can cause SJS/TEN.
5 A's a. Antibiotics- septra, B-lactams b. Anti convulsants – carbamazepine, VPA, phenytoin, phenobarb, lamotrigine c. Anti-inflammatory - NSAIDS d. Anti-retrovirals- abacavir, nevirapine e. Allopurinol
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What are 7 components of the SCORTEN score? (include specific values)
CA2B3S CAABBBS Cancer Age >40 Area of body > 10% Bicarb <20 BUN >10 Beats per minute >120 Sugar >14
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15. What are 5 medications that cause fixed drug eruption?
PAST Psuedoephedrine Anti-inflammatories: ASA, saids, acetaminophen, naproxen Sulfonamides Tetracycinlines
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What are 5 conditions associated with photoaging
a. Solar elastosis, cutis rhomboidalis nuchae b. Poikiloderma of civatte c. Favre racouchot syndrome d. Colloid millium e. Erosive pustular dermatosis
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List 5 photodermatoses.
PMLE Solar urticaria Actinic prurigo Hydro vacciniforme Actinic dermatitis
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What are 3 cutaneous forms of amyloid? Which one has the highest risk of progression to systemic amyloidosis? Which one overlaps with notalgia paresthetica?
lichen Amyloid Macular -notalgia Nodular-highest risk, 7%
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What are 5 categories of triggers for CSVV
Infection- strep, hep C>B Autoimmune Malignancy Drug Other-cryoglobulinemia, emboli, thrombi
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List 5 causes of urticarial vasculitis
a. AI-CTD: lupus, sjogrens b. Drug – Nsaids, MTX, TNF alpha, cimetidine, fluoxetine, SSKI c. Infection-viral (hep) d. Malignancy -leukemia/lymphoma, malignancies e. Other-serum sickness
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21. List the systemic findings and symptoms of Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatous with polyangiitis. Which ANCA type is associated with each? GPA
i. Nasal/sinus: sinusitis, rhinorrhea, epistaxis ii. Pulmonary: cough, hemoptysis, SOB iii. Renal: GN w/ hematuria iv. Other: Arthralgias, ocular findings v. CNS: Peripheral neuropathy, CVA vi. Skin: Palpable purpura, PG-like nodules and ulcers, gingival hyperplasia with strawberry gums, oral ulcers vii. ANCA (PR3, C-ANCA)
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21. List the systemic findings and symptoms of Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatous with polyangiitis. Which ANCA type is associated with each? EGPA
i. Nasal/sinus: Allergic rhinitis, nasal polyps ii. Pulmonary: Asthma iii. Cardiac: cardiomyopathy, valvular disease iv. GI: N/V abdo pain v. Renal: Limited vi. CNS: mononeuritis multiplex vii. Cutaneous: Palpable purpura, painful subcutaneous nodules viii. Labs: Peripheral Eos ix. ANCA: P-ANCA > C-ANCA
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List the systemic findings and symptoms of Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatous with polyangiitis. Which ANCA type is associated with each? MPA
c. MPA i. Pulmonary: Alveolar hemorhage ii. Renal: GN iii. Neuro: Neuropathy, mononeuritis multiplex iv. Cutaneous: Palpable purpura, livedo reticularis, ulcers, retiform purpura v. ANCA: (MPO, P-ANCA)
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List 5 pathology features of eczematous dermatitis.
a. Focal parakeratosis b. Acanthosis if more chronic c. Epidermal spongiosis (hallmark) d. Intraepidermal vesicle and bullae formation if acute e. Superficial perivascular lymphocytic infiltrate f. Variable eosinophilic infiltrate
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2. List 7 pathology features of lichen simplex chronicus.
a. Hyperkeratosis b. Focal parakeratosis c. Impressive irregular acanthosis d. Hypergranulosis e. Vertical orientation of collagen in the dermal papillae f. Perivascular lymphocytic infiltrate g. Prominent fibroblasts, occasionally multi-nucleated fibroblasts (Montogmery giant cells) h. Enlarged nerves
233
List 6 pathology features of pityriasis rosea
SAMPLER- spongiosis, acanthosis, mounded parakeratosis, perivascular lymphocytes, extravasated reds a. Mounds of parakeratosis b. Occasional mild acanthosis c. Mild epidermal spongiosis d. Occasional dyskeratotic keratinocytes e. Superficial lymphocytic perivascular infiltrate f. Eosinophilic infiltrate sometimes present g. RBC extravasation in dermis
234
List 7 pathology features of psoriasis.
a. Confluent parakeratosis, hyperkeratosis b. Neutrophilic abscesses in the stratum corneum (Munro microabscesses) c. Neutrophils in spinous layer (spongiform pustules of Kojog) d. Regular acanthosis e. Elongated and clubbed rete ridges f. Hypogranulosis g. Suprapapillary thinning h. Dilated capillaries i. Superficial perivascular lymphocytic infiltrate
235
List 5 pathology features of PRP
a. Follicular plugging b. Shoulder parakeratosis-adjacent to follicular plugs c. “Checkerboard” parakeratosis alternating with orthokeratosis d. Irregular acanthosis e. Acantholysis, focal, occasionally f. Perivascular lymphocyte 2. Shortened, fat rete pegs 3. Dilated hair follicles 4. Follicular plugging 6. Hypergranulosis Focal acantholysis with dyskeratosis
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List 7 pathology features of Lichen Planus.
a. Compact hyperkeratosis, usually no parakeratosis b. Acanthosis-irregular c. Wedge shaped hypergranulosis d. Saw tooth rete ridges e. Lymphocytic infiltrate at DEJ (lichenoid) f. Basal cell layer liquefactive degeneration g. Colloid bodies often h. Melanin incontinence i. DIF reveals IgM, complement, fibrin staining of colloid bodies
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List 3 pathology features of lichen nitidus
a. Epidermal atrophy, parakeratosis, overlying ball of papillary dermal lymphocytes, with epidermal rete ridges forming collarette = “ball in clutch” b. Focal liquefactive degeneration of basal layer c. Multinucleated giant cells
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List 5 pathology features of PLEVA
P-parakeratosis focally w/ scale crust Lichenoid infiltrate w/ basal cell layer vaculoization, lymphocytic exocytosis into the epidermis Extravasated reds V-shaped/wedge shaped infiltrate A-apoptotic KCs S-Spongiosis w/ epidermal vesicles and papillary dermal edema
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List 3 pathology features of urticaria.
a. Normal epidermis b. Papillary dermal edema c. Sparse perivascular and interstitial infiltrate of eosinophils, lymphocytes, neutrophils and/or mast cells
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List 6 pathology features of Erythema multiforme.
a. Basal layer degeneration, sometimes subepidermal bullae b. Perivascular and interface infiltrate of lymphocytes, sometimes eos c. Necrotic keratinocytes d. Spongiosis, rarely intraepidermal vesicles e. Papillary dermal edema f. Extravasated RBCs, sometimes
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List 5 pathology features of sweet’s syndrome.
a. Epidermal necrosis b. Superficial derma edema, sometimes subepidermal blister c. Diffuse dermal neutrophils (also lymphocytes, histiocytes, eosinophils) d. Nuclear dust, no true vasculitis e. Sometimes extravasated erythrocytes
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List 6 pathology features of erythema ab igne.
a. Epidermal atrophy b. Keratinocyte atypia c. Basal cell layer degeneration d. Dilated dermal blood vessels e. Elastosis in the dermis f. Melanin incontinence g. Hemosiderin the dermis
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13. List 5 pathology features of LCV.
a. Epidermis varies from normal to necrotic, sometimes with vesicles or pustules b. Fibrinoid necrosis of vessel wall c. Nuclear dust d. Superficial and mid perivascular infiltrate, predominantly neutrophils e. RBC extravasation f. Thrombi
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14. List 4 pathology features of granuloma annulare.
a. Normal epidermis b. Palisading granulomas (histiocytes) around foci of necrobiosis and mucin c. Often single filing or subtle interstitial pattern of histiocytes or giant cells between collagen bundle d. Perivascular lymphocytes
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List 3 features NLD
a. Epidermis normal, atrophic or ulcerated b. Palisading granulomas in the dermis, often oriented parallel to the epidermis, surrounding necrobiotic collagen, often with sclerosis c. Dermal interstitial infiltrate of histiocytes, multinucleated giant cells, lymphocytes, plasma cells
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16. List 3 pathology features of Erythema nodosum.
a. Septal panniculitis of lymphocytes, histiocytes, neutrophils b. Multinucleated giant cells in older lesions, without caseation c. Septal fibrosis in older lesions d. Mild fat necrosis sometimes with foamy histiocytes
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List 7 path findings MF
a. Atypical lymphocytes with angulated nuclei b. Haloed cells (perinuclear halo) c. Epidermotropism with minimal spongiosis d. Lymphocytes in epidermis larger than ones in dermis e. Lining up of lymphocytes along the DEJ f. Pautriers microabscesses-lymphocytes in epidermis g. Thickening of collagen in papillary dermis h. Lichenoid infiltrate
248
List 2 calcium stains
von kossa alizarin red Von kossa all read calcium
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List 1 iron/hemosiderin stain.
Prussian blue “Russia made of iron
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List 5 mucin stains
a. Alcian blue PH 0.5, 2.5 b. Colloidal iron c. Toluidine blue d. PAS e. Mucicarmine CAT-PM
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List 3 amyloid stains.
a. Congo red b. Thioflavin T c. Cresyl Violet
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List 3 fungal stains.
a. PAS b. PAS-Diastase c. GMS (Gormori methenamine silver)
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What stain is used for leprosy?
a. FITE FITE LEPROSY
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What stain is commonly used for acid fast bacteria?
a. Zhiel-Neelson
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What are 2 silver stains and what are they used for?
a. Gormori methenamine (GMS fungal) b. Warthin Starry Spirochetes-syphillis, borrelia
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26. Which stain is used for leishmania?
a. Giemsa We fite leprosy We get “GIET after” leishmania
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3 mast cells stains
toluidine blue, Giemsa, Leder Take M (for mast cells) to your Great Leader
258
What is the direct method? The indirect method? List the substrate and reagent for each and 3 examples of each
i. Direct: Look at patient’s tissue direct to see if ab or complement bound. 1. Substrate: Patient perlesional skin 2. Reagent: Fluorescently labeled anti-human IgM, IgG, IgA and C3 that bind to pathogenic ab’s attached to skin targets 3. E.g. DIF, salt split skin with DIF, direct immunoelectron microscopy, FOAM Indirect: Look at patients serum and see if ab’s present that bind to substrate 1. Substrate: Tissue (rat bladder, monkey esophagus, guinea pig esophagus) 2. Reagent: Patients serum w/ ab’s against antigen , then 2nd ab added that is anti human and fluorescently labelled 3. IIF, IIF with salt split skin, indirect immunoelectron microscopy, immunoprecipitation, immunoblotting, ELISA
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Where is the split in salt split skin
lamina lucida
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What are 4 reasons DIF can be negative despite the patient having the disease
Media expired Sat in media too long Leg lesion samples Perilesional or too far from lemon sampled
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d. How does Direct immune electron microscopy work
Patients perilesional skin mixed with anti human Fc ab’s labelled with gold , examined with electron microscopy
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What is FOAM
"Fluorescence overlay antigen mapping" Anti-human ab’s labelled with specific coloured label, also colour eith different label collagen typ 4, type 7, laminin 332, integrin Conofocal laser microscopy to examine
263
Name 5 types of substrate for IIF
i. Human skin ii. Guinea pig esophagus iii. Monkey esophagus iv. Rat bladder epithelium v. Keratinocyte culture
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Name the 4 intercellular junctions and what they anchor
Desmosomes- keratin Gap junctions Adherens junctions- actin Tight
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Name the 3 protein families that make up intercellular junctions
Cadherens: Desmoglein, desmocollin, cadherin Armadillo proteins: Plakoglobin, plakophilin, alpha beta catenin Plakins: Desmoplakin, periplakin, envoplaikin, BPAG1/2, pectin Connexins
266
From inside to out name the proteins in desmosomes
DSG/DSC Plakoglobin, plakophilin Desmoplakin
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From top to bottom name the proteins on hemi-desmosome
BPAG1/180, plectin Lamina lucida: BPAG2/230, integrin subunit alpha and beta Lamina densa: Collagen type IV, Laminin 332 Sublamina densa: Collagen type VII, collagen type II and type I, elastin
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Where is DSG-1 present vs. DSG-3 ? Explain DSG compensation
a. DSG1 present in skin, superficial>deep, less in mucosal. DSG-3 is deeper in epidermis and high prevalence in both skin and mucosa b. Compensation: If DSG3 is lost in skin, DSG1 can compensate, but NOT in mucosal surfaces. If DSG-1 is lost, DSG3 cannot compensate. SO, PF always skin, but PV is sometimes only mouth, with skin in 50-80% i. *Can get epitope spreading from ab to DSG 1 to DSG 3 so PF PV
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DSG-1 affected causes?
PF
270
DSG-3 affected causes?
PV-mucosal
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DSG-1/3 affected?
PV-mucosa and skin
272
What is the ab in pemphigus
IgG4
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Where is the split in PF? PV?
PF --> granular layer PV--> spinous layer
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List 5 pemphigus variants
Pemphigus Folicaceous: PF IgA pemphigus foliaceous Fogo selvagem Pemphigus erythematous Pemphigus herpetiformis Drug induced PF
275
List the 5 PV variants
i. PV: Mucosal (DSG3), Mucocutaneous (DSG 1/3) ii. Pemphigus vegetans-Neuman, Hallopeau iii. Paraneoplastic (plakins, cadherins) iv. IgA pemphigus/Intraepithelial neutrophilic (DSG 3, DSC) v. Drug induced PV vi. Pemphigus herpetiformis (DSG 1>3)
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How does PF present
a. Well demarcated crusted erosions, impetigo like on erythematous base in seborrheic areas, “cornflake” like scale, + nikolsky, no oral
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What is the difference between thiol and non-thiol drugs
b. Most induced by thiol drugs (e.g. sulfhydryl containing), which can cause DIRECT acantholysis (Penicillamine, ACE, ARBs) --> MORE PF LIKE c. Non-thiol drugs can induce an immune phenomenon causing more a PV presentation -B, B, C, G PR (Beta lactams, gold, CCB, B-blocker, piroxicam, rifampin)
278
Name 3 drugs that can cause drug induced PF
penicillin, penicillamine, captopril
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How does pemphigus herpetiformis present
polycyclic urticarial plaques, ocasional herpetiform vesicles, minimal acantholysis
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Which substrate do Ab’s bind best to in IIF for PF? PV? PNP PV
PF--> guinea pig esophagus PV--> monkey esophagus PNP PV--> rat bladder
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13) What is the difference between pemphigus vegetans of hallopeau vs. neumann
Neumann-flaccid bullae and erosions that develop vegetating plaques, similar course to PV Hallopeau --> Crops of pustules turn into crusted plaques. May remit spont.
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14) List 10 clinical differences that PNPV differs from PV
a. Polymorphous rash (PV, PF, lichenoid, GVHD, BP) > flaccid bullae/oral erosions b. Vermillion lip border involved> buccal mucosa c. Extensive erosive stomatitis d. Scalp usually spared e. Palmar/plantar involvement>palms/soles spared f. Non scarring conjunctivitis > periocular involvement g. Asboe hansen/nikolsky usually negative h. Suprabasal acantholysis + lichenoid, subepidermal bullae i. IgG and C3 intercellular and linear at BMZ > only intercellular j. Indirect IF on rat bladder possibly positive vs. negative in PV i. Helpful to use rat bladder epithelium, should be + in PNP and negative in PV ii. Contains plakins but NO desmogleins k. Bronchiolitis obliterans > no systemic l. Mortality 90% >minimal mortality m. Fails typical PV therapies
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What are the 5 different presentations (based on B and T-cell predominance) of PNPV a
B-cell: pemphigus like BP-like EM like GVHD like LP like T-cell
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5 malignancies associated with PNPV
a. CLL b. Non hodgkin lymphoma c. Castlemans d. Thymoma e. Sarcoma f. Waldenstroms g. Adenocarcinomas-breast, prostate, lung , pancreas h. *Lichenoid variant usually Castlemans
285
17) Name 6 antigens seen in PNPV
Plectin, periplakin, envoplakin, desmoplakin, BPAG1, DSG-3, 1
286
why does rat bladder help distinguish PNPV
contains plakins and no DSGs
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19) What type of B cell is typically producing abs in PV/PF
B lymphocytes > long lasting memory cells
288
First line treatments for PF/PV
Prednisone alone Pred + ritux Prednisone + steroid sparing- AZA or MMF
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What is the rituxumab dosing for PV
1 gram day 1 and 14, 500 mg at month 12 and 18
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Treatment of choice pemphigus herpetiformis
Dapsone
291
List 2 epidermal and 10 sub epidermal blistering d/o
a. PF and variants b. PV and variants c. Paraneoplastic PV 1.Bullous pemphigoid 2. Pempigoid/herpes gestationis 3.Anti-laminin gamma1 pemphigoid 4. Mucous membrane pemphigoid (MMP), 5. Ocular predominant MMP 6. Anti-Laminin 5 MMP 7. Dermatitis herpetiformis 8. Linear IgA disease 9. EBA 10. Bullous SLE
292
List the variants of Bullous Pemphigoid per Scott Walsh- there are 20
Localized: 1. Pretibial 2. Dyshidrosiform 3. Stomal 4. Radiation aggravated 5. Hempiplegic (stroke site) 6. Stump 7. Vulvar 8. Umbilical Generalized: 1. Urticarial (classic) 2. Eczematous 3. Pemphigoid nodularis 4. Invisible 5. Erythrodermic 6. Vegetans 7. Seborrheic 8. TEN-like 9. Erosive 10. Ecthyma gangrenosum like 11. Palmar-plantar purpuric 12. Lichen planus pemphigodes 13. Neonatal 14. childhood 15. drug induced
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Name 4 causes pemphigoid gestations/herpes gestations and 2 associations
PRegnancy Choriocarcinoma HYatidiform mole Trophoblatic tumor Association HLA DR4, thyroid dz
294
When does herpes gestationis appear in pregnancy? Where does it occur?
2nd trimester > 3rd, improves with delivery then flares Periumbilical- involves the umbilicus
295
Name 3 reasons for pemphigoid gestationis to recur
subsequent pregnancy with same dad ocp menses
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27) Name 3 treatments for pemphigoid gestationis
a. Pred, CsA, IVIG
297
28) What is the autoantibody and antigen in BP
BP180 (BPAG2)* and BP230 (BPAG1) IgG1 against NC16A of BPAG-2 (180 or COL17) Can see IgG4 against BPAG1/230 but non pathogenic
298
What % BP have mucous membrane involvement
10-20%
299
What is most common location BP
pretibial
300
3 diseases associated with BP
MS Parkinsons stroke dementia epilepsy
301
5 drugs that cause BP
Enalapril Furosemide Spironolactone Gliptins PD-1
302
Name 5 biochemical requirements other than Ab’s for BP disease
a. Mast cells b. Complement c. Granulocytes-eos, neuts d. Gelatinase causes blister e. IgE then class switch to IgG (prevent conversion, prevent disease?)
303
Name 4 methods of treating BP, broadly
a. Inhibit auto-antibody production: Pred, MMF, AZA, etc. b. Catabolize autoantibodies: IVIG, omalizumab c. Inhibit mast cells: Cetirizine, omalizumab d. Inhibit inflammatory infiltrate; dapsone, sulfasalazine, colchicine ,doxycycline, nicotinamide
304
35) What is Anti-Laminin Gamma 1 Pemphigoid? Name its other name its target, its clinical presentation, path differences/SSS, tx differences
Anti p200 pemphigoid 200 kd antigen in lamina lucida, which is the C-terminus of laminin 1 gamma in deep lamina lucida a. Anti-P200 pemphigoid b. Antigen: C-terminus of laminin 1 gamma in 90% (DEEP laminin) c. BP like eruption in males, younger (50-70), concomitant psoriasis, have palmar-plantar involvment, mucosal erosions, milia and scarring can occur d. Floor of SSS, more neutrophilic infilitrate e. Tx: Dapsone, sulfasalazine, colchicine
305
How to tell the difference MMP with skin involvement vs. BP with mucosal involvement
a. No OCULAR involvement in BP
306
How does MMP differ from BP
a. Predominantly mucosal, can have ocular, tendency to scar, chronic course b. Deeper antigenic targets generally c. A disease phenotype
307
38) What are the antigenic targets in MMP
i. Lamin 332 ii. BP-180 C-terminus, BP 230 iii. Integrin iv. Type 7 collagen
308
39) List 3-5 variants of mucous membrane pemphigoid and their antigenic target (includes another scarring that could be on its own)
a. Anti-Epiligrin MMP/Anti-laminin 5/Laminin 332 cicatricial pemphigoid b. Pure ocular pemphigoid (B4 integrin) c. Classic form: BP-180 C terminus d. Variable mucosal but no skin involvement e. Brunsting perry variant f. Anterior oral pemphigoid Laminin 332 (Anti epiligrin) BP-180 C-terminus (classic) Integrin (pure ocular) Collagen 7?
309
40) What are is the most common site of MMP and 3 other sites,
a. Oral in 85%  gingival, buccal, palate b. Conjuctiva blindness c. Esophagus  can lead to strictures, resp distress d. Genitals anal, vaginal
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TOC for anterior oral MMP
dapsone
311
Treatment for MMP if ocular, airway or genital dz
Pred cyclo leflunomide or MMF
312
What are the 3 types of LABD
Adult LABD -laminda lucida - BP like -sublamina densa- EBA like CBDC: chronic bullous disease of childhood Drug induced
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44) Name 4 drugs that can induce LABD
vancomycin, penicillin, cephalosporins amiodarone sulfamethaxazole C-VAPS
314
Which antigens targeted in LABD
Shed ectodomain of BP-180 Split products of BP180--> 120 KD= LAD1 or 97 KD LAd97 Collagen 7 in LABD like
315
3 tx for LABD
Dapsone Colchciine Sulfasalzine
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NAme 4 ddx for neutrophilic sub epidermal blistering diseases
LABD DH Anti laminin gamma 1 bullous SLE
317
Name two variants of acquired EBA
a. Classic mechanobullous EBA (non-inflammatory-acral blisters, scarring, milia, mitten deformities, pauci inflammatory) supress ab>inflammation b. Inflammatory (BP-like) EBA  flexural/intertriginous bullae that heal w/out scarring, eosinophils, misinterpreted often c. Inflammatory EBA (CP-like) brunsting perry w/ scarring alopecia, may have scarring oropharyngeal,
318
49) What is EBA associated with?
chrons asians
319
Antigen target EBA
a. Collagen 7
320
4 diagnostic criteria for bullous SLE
a. Diagnosis of SLE b. Vesicles/bullae arising on, but not limited to, sun exposed skin c. Subepidermal blistering w/ predominant neutrophils d. DIF showing linear or granular IgG (90%), C3 (90%) or IgM (60%), IgA (60%) in the BMZ.
321
TOc bullousSLE
dapsone colchicine sulfa
322
53) What is the clinical presentation DH 54) What is antigen target in DH? What is target in gut?
a. Grouped herpetiform and urticarial and excoriation on buttocks, knees, elbows IGA to TTG-3 in skin TTG 2 in gut
323
55) What do we see on path for DH
a. Subepidermal blisters/absceses at the dermal papillae tips with ++ neutrophils b. “Squirting papillae”
324
What do you order for a celiac profile
a. IgA and IgG to: Gliadin, TTG, EMA b. IgA level c. Biopsy and DIF
325
57) Describe the antigen, DIF pattern and salt split skin pattern for: PF, PV, PNPV, pemphigus erythematous, IgA pemphigus, BP, pemphigoid gestations, MMP, laminin 332, DH, LABD, EBA, bullous SLE
PF/PV: DSG1/3, linear IgG +-C3, intercellular IgA pemphigus: DSC1 (SPD), DSG1/3 (IEN), Linear IgA intercellular Pemphigus erythematous: DSG1/3, Granular to linear IgG and C3 PNPV: Antigen: Many-plakin family, DSG1/3, BPAG180, plectin, Linear IgG/C3 intercellular and BMZ BP: BPAG2/180, linear IgG/C3, n serrated, roof salt split Pemphigoid gestationis: Linear C3 MMP: laminin-5, ocular, classic, variable mucosa -DIF for all: Linear IgG and C3 Salt split skin: -laminin-5: floor -ocular: roof or both -classic: roof or both -variable mucosal: roof or both EBA: Linear IgG > C3 Salt split: Floor Bullous SLE i. DIF: Linear IgG, C3, can also see IgM, IgA 1. *Can also see granular IgG, M, A, C3 =lupus band ii. Salt split: Floor LABD i. DIF: Linear IgA ii. Salt split: Roof or floor DH i. DIF: Granular IgA in dermal papillae
326
Ab target for bullous impetigo
DSG 1
327
Ab target for SSSS
DSG 1
328
Ab target for PF
DSG 1
329
Ab target for PV
DSG3, 1
330
IgA pemphigus
DSC- SPD DSG1/3 - IEN
331
Ab for pemphigus herpetiformis
DSG1 >3
332
6) PV:
6) PV: DSG3 or DSG1+3,
333
BP
BP180/BPAG2-> N16CA domain non collagenous
334
Pemphigoid gestationis
BPAG 180/2
335
LABD
LAD-1 (120 KD cleaved portion of BP180 antigen) and LABD97 (97 Kd portion of LAD-1)
336
MMP antigen
BP180 c-terminus
337
Ocular predominant
B4 integrin
338
Anti-epiligrin
Laminin 5/332/Epiligrin
339
13) p200 pemphigoid:
Laminin gamma 1
340
EBA
Collagen 7
341
Bullous SLE
Collagen 7
342
Where does the split occur in the following conditions: a) EB simplex; b) Junctional EB; c) Dystrophic EB.
EB simplex: Intraepidermil JEB:lamina lucida DEB: sub lamina densa Kindler: Mixed
343
Which protein is mutated in each of the following conditions (this is on your exam): EBS localized, generalized intermediate, generalized severe, mottled-pigmentation EBS w/ muscular dystrophy JEB JEB w/ pyloric atresia Dominant DEB Recessive DEB Kindler
EBS localized/generalized intermediate/generalized severe/mottled-pigmentation: KRT5/KRT14= Keratin 5 and 14 EBS with muscular dystrophy: PLEC- Plectin Junctional EB – generalized severe/generalized intermediate: LAMA3/LAMB3/LAMC2/COL17A1- Laminin 332 and Collagen XVII (BPAG2/180) JEB with pyloric atresia: ITGA6/ITGB4- Alpha-6 Beta-4 integrin o 15% plectin mutation Dominant Dystrophic EB: COL7A1- Collagen VII (missense mutation) Recessive Dystrophic EB: COL7A1- Collagen VII (premature termination =complete lack anchoring fibrils) Kindler Syndrome: FERMT1- Fermitin family homolog 1
344
What disease states associated with: BPAG1
BP EBS
345
What disease states associated with: BPAG2
N16A terminus: Bullous pemphigoid, Pemphigoid gestationis, LABD Carboxy terminus: Mucous membrane pemphigoid
346
alpha6-beta4 integrin:
JEB w/ pyloric atresia Ocular MMP (B4 subunit)
347
Laminin 332/Laminin5/Epiligrin
Anti epiligrin MMP
348
Plectin
EBS w/ muscular dystrophy, EB w/ pyloric atresia -15%
349
Type IV collagen
Goodpasture Alport syndrome
350
Collagen 7
EBA Bullous SLE dystrophic EB
351
Which disease states are associated with mutations in the following: Keratin 1
i. Epidermolytic ichthyosis ii. Epidermolytic and non-epidermolytic PPK iii. Icthyosis hystrix of curth-macklin
352
Which disease states are associated with mutations in the following: Keratin 2
Superficial epidermolytic icthyosis
353
Which disease states are associated with mutations in the following: Keratin 4
white sponge nevus
354
Keratin 5
EBS Dowling degos
355
Keratin 6a/keratin 16
Pachyonychia congeenita type I
356
Keratin 6b/17
Pachyonychia congeenita type II
357
Name the 5 indolent primary cutaneous T cell lymphomas
MF Primary cutaneous CD30+ lymphoproliferative d/o -Anaplastic large cell lymphoma -Lymphomatoid papulosis Subacute panniculitis-like T-cell lymphoma EBV+ lymphoproliferative disorder in childhood -Hydro vacciniforme like LPD -Hypersensitivity reations to mosquito bites Primary cutaneous peripheral T-cell lymphoma, rare subtypes Primary cutaneous CD4+ small/medium t-cell LPD Primary cutaneous Acral CD8+ T-cell lymphoma
358
Name the 5 aggressive CTCLs
1. Sezary syndrome 2. Extranodal NK/T-cell lymphoma, nasal type 3. aggressive epidermotropic cd8+ cytotoxic CTCL 4. Gamma-delta T-cell lymphoma 5. peripheral T-cell lymphoma NOS
359
What are the 3 known subtypes of MF? Which are aggressive, which indolent
Granulomatous slack skin Pagetoid reticulosis Folliculotropic-aggressive w/ 75% 5 yr survival
360
What are the 2 variants of folliculotropic MF? Which has worse prognosis
Acneiform/cyst like = worse prog Follicular papules
361
4) Where is the classic location for pagetoid reticulosis? What is the life expectancy? How does it present
Hands, foot (bottom), face 100% Solitary plaque, psoirrasiform, slow growing "worringer dollop"
362
What is the variant of pagetoid reticulosis that can be systemic and aggressive
Ketron Goodman
363
What is granulomatous slack skin associated with in 1/3 of cases
Hodgkin's lymphoma
364
What is common but least specific cd change in MF
CD7 loss
365
What is the specific change in CD, MF
Loss of CD5 and 2
366
Is the typical pattern of CD in MF, what is the exception
CD3+ CD4+ CD8- CD30- hypopigmented MF tends to be CD8+. CD4-
367
9) How does the ratio of CD4:CD8 change as MF progresses
increases
368
10) What is the diagnostic criteria of Sezary syndrome
Ertyrhoderma >80% TCR clonality >1000 sezary cells/ul -CD4:8 ratio >10 -Abberant expression T-cell antigens (loss of t-cell markers like CD7 and CD26 most commonl)
369
Name 11 morphological variants MF
Hypopigmented Hyperpigmented Poikilodermatous Telangiectatic Atrophic Acral Palmoplantar Lichenoid Granulomatouos pigmented purpuric dermatosis Purpric
370
Name 10 morphological variants MF
Hypopigmented Hyperpigmented Poikilodermatous Telangiectatic Atrophic Purpric Acral Palmoplantar Lichenoid Granulomatouos pigmented puru0ric dermatosis
371
What is the staging for MF
Stage IA- patch or plaque <10% BSA Stage IB-patch or plaque >10% BSA Stage IIA- palpable reactive lymphadenopathy, can have histological involvement but with normal architecture. Stage IIB- tumors w/up to palpable LN but no histo Stage III- Erythroderma with up to histo LN Stage IVA1- Sezary cells > 1000, any T, up to histo LN Stage IVA2- histologically involved nodes with architecture effaced, anyB Stage IVB-Mets, any B *All can have up to 1000 cells/ul and >5%, even stage IA
372
What is the 5 year survival based on stage of MF
1A- same as age match controls 1B/2A- 73% (>10%BSA/palpable lymphadenopathy) 2B/III-44% (tumor or erythroderma) IV-27% (Mets)
373
What is the best way to diagnose Sezary
Blood flow cytometry with TCR CT or PET +- LN biopsy
374
What are the two variants of extranodal NK t-cell lymphoma
BOTH EBV + a. Nasal type – “lethal midline granuloma” b. Extranasal type – skin, GI, spleen
375
What are 8 treatments for Stage IA/B, 2A disease
a. Topical steroids b. Topical retinoids c. Topical Imiquimod d. Topical Nitrogen mustard e. Phototherapy-PUVA, nbUVB f. Total skin electron beam g. Systemic +- phototherapy -Oral retinoid (alitretinoin) -Interferon + PUVA -Methotrexate + PUVA
376
What are 8 systemic therapies for Stage 2B and higher
*Phototherapy and TSEB as adjuncts b. Retinoids-Alitretinoin c. Targeted radiation d. Histone deacetylase inhibitors: i. Vorinostat (Zolinza), Romidepsion (Istodax) e. MTX f. Interferon g. ECP if circulating clone h. Chlorbuxil i. Brentximab vedotin Mogalizumab
377
21) What are the immune changes sen in progression of MF?
a. Increasing TH2 response, decreasing TH1 b. Dsyregulated cytokine production and inability to fight infection c. Accumulation genetic mutations and epigenetic changes in lymphoma cells, possibility of large cell transformation
378
Most common cause death MF
sepsis
379
23) Name 8 anti-itch therapies for MF
a. Camphor/menthol b. Pramoxine c. Doxepin (TCA) d. Mirtazapine e. Gabapentin/pregabalain f. Naltrexone g. Butorphanol h. Aprepitant
380
What is the morphological change on histopathology seen in large cell transformation? What is a clinical sign of this change? What is median survival post LCT?
Small/medium sized cerebriform cells->CD30+ or CD30- large cells 4x size make >25% of total lymphoid infiltrate ulceration of tumor or nodule 12-22 months
381
What is TOC for PC CD4+ small/medium pleomorphic T-cell lymphoproliferative d/o
surgical excision or radiation
382
How does CD8+ acral T-cell lymphoma present
a. Slowly progessive papulonodules- ear>nose>foot
383
How does CD4+ small/medium pleomorphic T-cell lymphoproliferative d/o present
b. Presents as small solitary plaque head/neck
384
27) What is the Sezary triad
LAD erythroderma sezry cells
385
28) What is the cell type in Sezary
CD4+ CCR7+
386
29) Name 9 histopath signs of MF
a. Atypical lymphocytes with angulated nuclei b. Lymphocytes lined up at DEJ c. Epidermotropism d. Haloed cells e. Minimal spongiosis in relation to the lymphocytic epidermotropism f. Darier-Pautrier’s micro abscesses g. Lymphocytes in epidermis larger than demis h. Thickening of collagen in papillary dermis i. Lichenoid infiltrate
387
30) Name 7 conditions where CD30+ cells can be seen
a. Reactive: arthropod bite, viral (HSV, orf), infestations, drug reaction b. Malignant-primary cutaneous: i. LyP, Anaplastic large cell lymphoma ii. MF large cell transformation, Sezary, pagetoid reticulosis, extra-nodal NK/T cell , peripheral T-cell lymphoma c. Malignant-systemic: HODKGINS lymphoma, systemic anaplastic large cell
388
31) Name 3 CD4 markers in LyP
CD30+, CD4+, CD7 decreased
389
32) Name 3 conditions associated with LyP
a. MF (75%) b. Hodgkins (1%) c. Systemic anaplastic large cell lymphoma (24%) 20%* have preceding, concurrent or subsequent lymphoma
390
Name 7 treatments for LYP
a. Observation b. Topical steroids-class I/II -usully not helpful c. ILK d. Phototherapy- PUVA e. Imiquimod f. MTX- helpful g. MMF h. doxycycline -usually not helpful Aggresive would be systemic chemo or TSEB topical mechlorethamine or carmustine, and low-dose etoposide. Excision, radiation
391
35) How does LyP present? Anaplastic large cell lymphoma?
a. LYP: recurrent crops ulcerated red-brown papulonodules, usually multiple (40ish), heal with varioliform scars, self resolve then recur b. Anaplastic large cell: single rapidly growing ulcerated nodule, doesn’t come and go,
392
36) Name 2 differences between primary and secondary anaplastic large cell lymphoma
Secondary evolves from another lymphoma - Secondary often has T2;5 ALK translocation -secondary is EMA positive with ALK expression -primary better prognosis *must exclude MF in transformation
393
Treatment for CD30+ ALCL
brentuximab
394
37) Hydro vacciniforme-like LPD (CD8+) and hypersensitivity reaction to insect bites (EBV related lymphoproliferative d/o) (NK-type, CD56+) are associated with increased risk of what?
Systemic EBV+ NK or T-cell lymphoma
395
What is subacute panniculitis like T-cell lymphoma often signed out as
lobular lymphocytic ppanniculitis confused often wth lupus panniculitis
396
how does primary cutaneous CD4+ small/medium pleomorphic T cell lymphporpilferatice d/o present? Name 3 T-cell markers
solitary plaque on head/neck trunk CD4+. BCL6+. CXCL13+
397
What are the T-cell markers in subacute panniculitis like t cell lymphoma
CD4 -, CD8+, TIA+, granzyme B+ with alpha/beta phenotype CD56-,
398
T-cell markers in cd8+ acral
CD8+, TIA+, granzyme and perforin neg
399
What % of cutaneous lymphomas are B-cells
20-25%
400
What are the 5 L's
lupus tumidus pmle jessners lymphoma psuedolymphoma
401
Name the 5 major types of primary cutaneous B-cell Lymphoma
a. Primary cutaneous follicle center lymphoma (PCFCL) b. Primary cutaneous marginal zone B cell lymphoma (PCMZL) i. Includes previously called plasmacytoma or immunocytoma c. Primary cutaneous diffuse large b-cell lymphoma, leg type (PCDLBCL, LT) d. Intravascular large B-cell lymphoma e. Diffuse large B-cell lymphoma, NOS
402
Name 5 less common B-cell lymphomas
a. B-cell lymphoma with secondary cutaneous involvement b. Precursor B-cell lymphoblastic lymphoma/leukemiakids/young adults c. EBV-associated DLBCL of elderly d. Immunosuppressive induced lymphoproliferative disorders e. EBV mucocutaneous ulcer (often immune suppressed)
403
43) Which B-cell lymphomas are aggressive? What are their 5 year disease specific survivials
intravascular diffuse
404
45) What is the most common to least common B-cell lymphoma
a. Most common: PCFCL>PCMZL >LT > EBV = intravascular
405
46) Which is the best lab predictor of systemic lymphoma
LDH
406
48) What is the clinical presenation of PcFCL. Who gets it?
H/N of middle age patients (60s) Solitary often Male=Female Pink/plum nodules Sometimes peripheral patch erythema (Crostis), sometimes acneiform or agminated papules nearby.
407
What is Crust's lymphoma
a. Primary cutaneous follicular center lymphoma w/surrounding erythematous patch, papules and plaques
408
51) What are 3 histologic patterns in FCL
a. Follicular b. Follicular diffuse c. Diffuse (must differentiate DLBCL-LT)
409
52) What is the status of CD20, BCL6, CD10, BCL-2
a. CD20 +, BCL6+, CD10 + (follicle cell markers), BCL-2 -
410
53) Who gets PCMZL?
Male predominant 40s
411
What is clinical presentation of PCMZL
a. Pink-violet or red-brown papules, plaques nodules b. Often multifocal c. upper extremities (Arms, trunk, head, neck)>lower d. Asymptomatic, indolent, no B-symptoms, almost never progresses
412
55) What are the two distinct entities of marginal zone lymphoma
1. Express class switched Igs: More benign i. IgA, G, E, ii. Show many T-cells, no risk transformation into diffuse large b-cell, thought to be more lymphoid hyperplasia iii. Do not express CXRC3 2. Non class switched : More aggressive Express IgM and often CXCR3 positive ii. Share features with MALT lymphoma iii. More like to have extracutaneous disease iv. Risk transformation to DLBCL
413
56) Name 4 cell markers in MZL
CD20+, BCL2+, CD10-, BCL6-
414
57) What is the cell type characterized by in DLBCL-LT.
a. Centroblastic and immunoblastic
415
58) What are the cell markers for DLBCL-LT: CD20, BCL2, BCL6, CD10, MUM-1, FOX-P!?
a. Positive for CD20, BCL-2, MUM-1, FOX-P1, +- BCL6, CD10- *BCL-2 + and MUM-1
416
59) What is the presentation of primary cutaneous DLBCL-leg type
a. Primary elderly women b. Solitary or clustered red-brown nodules on distal lower leg, uniltateral, often ulcerate c. *20% may arise other places, but still call leg-type
417
60) What is the 5 yr survival for DLBCL-LT
56%
418
Name 4 poor prognostic factors for DLBCL-LT
Both legs Multople tumors apoptotic cells Loss p16
419
62) What is presentation IVDLBCL
a. Painful telangectatic nodules or subcutaneous nodules b. *Must differentiate from secondary disease
420
63) What are 5 other skin lesions that can be seen in up to 1/3 of systemic IV lymphomas>? 3 lab findings?
Livedo Purpura Nodules Panniculitis Painful telangiectasis Anemia, LDOH, inflammatory markers
421
64) What are the 2 major forms of intravascular large B cell lymphoma
Western form--> skin/cns or skin only Asian--> multiorgan and HLH
422
65) What is the 5-year survival fro primary vs. Systemic intravascular lymphoma
a. 72% primary vs. 33% systemic
423
66) What is lymphocytoma cutis/psuedolymphoma/cutaneous lymphoid hyperplasia? Name 5 triggers. Where does it occur:
a. Benign reactive proliferation lymphocytes which mimicks cutaneous lymphoma clinically, thought to be from antigen stimulation b. arthropod bites, tick bites, contact allergen (gold, nickel, cobalt), vaccinations, tattoo allergens, drugs (phenytoin, MTX, clozapine, doxepin) c. face, earlobes, nipples, and scrotum
424
67) What % of cutaneous lymphoid hyperplasia will transform to lymphoma? Clonal vs. non?
a. Clonal: 25% b. Non clonal: 5%
425
69) Name 6 treatments for low grade B-cell lymphoma: MZL, FCL
69) Name 6 treatments for low grade B-cell lymphoma: MZL, FCL a. Active non-intervention b. ILK c. Surgical excision d. Radiation f. Subcutaneous/intralesions interferon-alpha-2a (e.g. multifocal lesions) g. Rtixumab: Intralesional (few localized) OR systemic (many)
426
70) What is TOC for PCMZL
surgical
427
71) What is a risk of radiation in follicle centre lymphoma
a. Recurrences in the field not included, eg. Crostis lymphoma  need 10-20 cm
428
What is TOC in PCDLBCL-LT and IVDLBCL
R-CHOP
429
73) Name 5 plasma cell dyscrasias
a. MGUS b. Smoldering myeloma c. Multiple myeloma d. Light chain deposition disease e. Amyloidosis
430
74) What are the main symptoms of MM: CRAB
a. Hypercalcemia b. Renal dysfunction c. Anemia d. Bone-pain and lytic>sclerotic lesions
431
Name 2 conditions where there is a proliferation of lymphoplasmacytic cells in the skin:
a. Extramedullary cutaneous plasmocytoma (marginal zone lymphoma now) b. Cutaneous Waldenstroma macroglublinemia (IgM monoclonal gammopathy present by definition
432
76) Name 7 conditions where there is deposition of monoclonal protein in the skin
a. Primary systemic amyloidosis-light chains b. Cryoglobulinemis occlusive vasculopathy (Type I) c. Follicular hyperkeratotic spicules d. Crystal storing histiocytosis e. Crystalglobulinemia f. IgM storage papules (cutaneous macroglublinosis) g. Subepidermal bullous dermatosis associated with IgM gammopathy
433
Name 5 conditions almost always asspociated with monoclonal gammopathy and 4 frequently associated
a. Always -Scleromyxedema -POEMS syndrome -AESOP: Adenopathy and Extensive skin patch overlying plasmacytoma -Schnitzler -Necrobiotic xanthrogranuloma d. Frequently i. Scleredema type 2 ii. Normolipemic plane xanthoma iii. Clarksons syndrome-idiopathic systemic capillary leak iv. Angioedema secondary to acquired C1 esterase inhibitor
434
78) Name 3 conditions associated primarily IgA gammopathy
a. EED b. Subcorneal pustular dermatosis c. Pyoderma gangrenosum
435
Name the Hanifin and Raja criteria
a. Need 3 major and 3 minor b. Major: Itch, Typical distribution, Chronic/relapsing dermatitis, Hx-fam or personal c. Minor: i. Early age of onset ii. Allergic salute iii. Allergic shiners iv. Cheilitis v. Anterior neck folds vi. Dennie morgan lines vii. Perifollicular papules viii. Keratosis pilaris ix. Xerosis x. Centrofacial paleness or erythema xi. White dermographism xii. Pityriasis alba xiii. Itchyosis/hyperlinear palms xiv. Hand/foot dermatitis xv. Nipple eczema xvi. Pruritus when sweating xvii. Elevated IgE xviii. Keratoconus xix. Anterior subcapsular cataract xx. Recurrent conjucitivtis xxi. Type I hypersensitivity/allergies xxii. Food intolerance xxiii. Intolerance to wood and lipid solvents xxiv. Course influenced by environmental and emotional factors
436
2. Name 6 variants of hand eczema
a. Dishydrotic b. Pompholyx c. Chronic fissured d. Hyperkeratotic e. ID reaction f. ICD g. ACD h. Pulpitis i. Nummular j. Interdigital
437
Name 3 reasons higher risk ACD in venous stasis
a. Broken skin barrier b. Chronic wounds c. Occlusive or semi-occlusive
438
Name top 9 categories ACD in venous stasis patients
a. Fragrances - balsam of peru, colophony b. Rubbers- carbamates, thiurams c. Abx – neomycin, bacitracin, fusidic acid d. Anti-septics – chlorhexidine, BPO, povidone e. Textiles and dyes f. Corticosteroids g. Preservatives- formaldehyde h. Vehicles – propylene glycol, lanolin i. Analgesic- esters like benzocaine
439
paraben paradox
a. ACD to paraben often tolerated unless skin is broken or compromised
440
6. How can you test if a product has nickel
Dimethyl dioxide
441
Allergen in poison ivy
urushiol
442
What are the 3 stages of pruritus pigmentosa
a. Early: Urticarial papules i. Neutrophilic spong b. Fully developed: crusted red papules, smooth papulo- vesicles or bullae i. Lymphocytic patchy interface pattern c. Late: Reticulated pigmentation i. Prominent melanophages
443
3 associations with prurigo pigmentosa
keto diet irritation from occluded swat ACD-nickel chrom textiles
444
10. What is the first treatment for pruritus pigmentosa
Doxy
445
Name 4 underlying causes/RF for asteatotic eczema
a. Nutritional deficiency e.g. vitamin A b. Aging-lose oil glands c. Hypothyroid d. Retinoids
446
Name 5 associations w/ nummular dermatitis
a. AD b. xerosis c. Id rxn to dermatophyte d. Id rxn venous stasis e. Id rxn ACD-nickel, PPD f. *often staph
447
13. Name 4 associations with prurigo nodules
a. CKD b. IDA c. AD d. ACD e. Insect bite reactions
448
14. What isMC cause gionotti crostti
a. EBV> CMV, enteroviruses, hep B, RSV
449
15. Compare and contrast swimmers itch vs. sea bathers eruption in terms of : Causative agent, distribution, time to onset, fresh vs. salt
i. Sea bathers:stings from nematocysts (stinging cells) from jelly fish and sea anemones ii. Swimmers itch: Parasitic flat worms called "shistomomes", their larval stage, go into skin and die b. Time course i. Sea bathers: 4-24 hrs, can have fever/systemic sx ii. Swimmers itch: 30 minutes c. Rash distribution i. Sea bathers: covered skin ii. Swimmers itch: exposed skin d. Salt or fresh water i. Sea bathers: salt ii. Swimmers itch: fresh
450
16. Causative agent CLM
Ancylstoma braziliensis
451
Compare and contrast HES subtypes
a. Lympho-small clonal pop’n lymphocytes making IL-5 or 13 driving EOS elevation, more dermatitic, more lung involvement b. Myelo- mutation (FIPL/PDGF translocation) causing proliferation in eosinophils, ulcerative lesions predominate, cardiac
452
Approach to urticaria from pathophys syandpoint
a. Immunologic i. Antibodies-CSU ii. IgE dependent- allergy (drugs, food, envrionemtnal, contact) iii. Immune complexes-serum sickness iv. Chronic infection H pylori, Hepatitis, other viral, bacterial parasitis v. hereditary fever syndromes vi. AI diseases b. Non immunologic i. Direct mast cell releasing agents- contrast, opiates ii. Exogenous- stinging nettle (contains histamine) iii. Leukotriene production -ASA, nsaids iv. Bradykinin production - ACE Physical/inducible (see below)
453
Approach to urticaria:
Acute < 6 weeks i. Allergic -drugs, food, environment, contact ii. Infection-MC viral iii. Serum sickness iv. Non immunologic (direct mast, external, leukotriene, bradykinin, contact-benzoic acid, balsam Peru Chronic > 6 weeks Immunologic: -Spontaneous 1. Autoantibody- CSU 2. Autoinflammatory- herecitary fever syndromes 3. Infections- HIV, hepatitis B/C, H.pylori, helminthic 4. AI disorders Non immunologic: Meds-Bradykinin, LKTRN Inducible 1. Aquagenic, solar, cold, cholinergic, delayed pressure, dermatographism, vibratory
454
3. Explain the difference between white and black dermographism
a. White – blanching response from vasoconstriction with stroking of skin, often in atopics b. Black-black/green colour on skin due to metal deposits on skin
455
What is the triple response of lewis
a. Red line – capillary dilation b. Wheal- edema from histamine release c. Flare – neurogenic response causing arteriolar dilation
456
5. Name 5 causes of contact urticaria
a. Latex b. PPD c. Benzoic acid d. PEG e. Parabens f. Exogenous-stinging nettle (non immunologic), sorbic acid, cinnamic aldehyde, balsam of peru
457
What is urticarial multiforme
a. Acute annular urticaria in children with preceding fever/cough, lesions < 2 hours b. Associated angioedema of hands, feet, no arthralgias/arthritis c. Dusky ecchymotic centre, no true target lesions, no mucous membrane lesions d. Dermographism often present e. Resolved 7-10 days f. Often responds to anti-histamines
458
Name 5 sources of bite reactions
a. Pediculosis pubis or capitis b. Cheyletiella (cat mites) c. Groups of 3 i. Fleas ii. Bed bugs iii. Cone nose bugs
459
Name 6 causes exaggerated bite reactions
a. CLL b. Mantle Cell lymphoma c. NK cell lymphoproliferative d. B-cell lymphoblastic lymphoma e. EBV f. Well’s g. HIV
460
What is papular urticaria
Insect, arthropod or parasite reaxctions that can persist e.g. post scabies
461
What is eosinophilic dermatosis of hematological malignancy (EDHM)
a. Edematous/urticarial papules, vesicles or bullae on patients with heme malig mimicking insect bite rxn b. Associated with: CLL, mantle cell lymphoma, ALL, AML, B-cell lymphoma, MM, MGUS,
462
Describe the 3 types of eosinophilic folliculitis
a. Classic- recurrent clusters of follicular papules and pustules on annular plaques, often in japanese b. Infancy associated – pustular scalp folliculitis c. Immunosuppression- intensely pruritic discrete edematous follicular papules w/ head and neck predominance often seen <250 CD4 or 3-6 months post HART
463
3 types of eosinophilic folliculitis
a. Classic- recurrent clusters of follicular papules and pustules on annular plaques, often in japanese b. Infancy associated – pustular scalp folliculitis c. Immunosuppression- intensely pruritic discrete edematous follicular papules w/ head and neck predominance often seen <250 CD4 or 3-6 months post HART
464
12. Name the stages of lyme disease and the rash associated
a. Early localized- erythema migrans b. Early disseminated Skin- erythema migrans chronicum (multiple erythema migrant spots), . Borrelia lymphocytoma is a rare presentation of early Lyme disease that has been reported in Europe. It presents as a nodular red-bluish swelling that usually occurs on the ear lobe or areola of the nipple. The lesions can be painful to touch. "SCORN: skin, cardiac, ocular, rheumatologic, neurologic" -CN palsies, meningitis, peripheral neuropathy, keratitis, AV block c. Chronic i. ANCS- arthritis, neuro, skin -arthritis, cognitive inmpairment, mononeuropathy, Skin in chronic 3. Acrodermatitis chronica atrophicans- reddish purple atrophic on dorsal hands/feet
465
13. What are the most common locations for scabies? What are the clinical presentations?
a. Webspaces, wrists, axillae, groin b. Crusted scabies, nodular scabies, papular urticaria c. *Often dermatitic and nodular
466
14. What are the 2 groups of urticarial vasculitis
a. Neutrophilic i. Hypocomplementic: Low C1q/C3/C4, high C1q antibodies, often associated w/ systrmic symptoms and other AI dz ii. Normocomplementimic: often post URTI, can also be seen with EBV, HEP B/C, serum sickness, NSAIDS/SSKI b. Lymphocytic
467
15. Name 4 associated diseases with Hypocomplementemic UV
a. Sjogrens, Lupus, paraproteinemias, multiple myeloma,
468
16. What syndromes can you see UV?
a. Muckle Wels- hereditary CAPS b. Cogans- hearing loss, systemic vasculitis, keratitis c. Schniztler- IgM paraproteinemia, fever, bone pain, d. Jacouds- UV with GN
469
17. What is acute hemorrhagic edema of infancy
a. LCV found in young children 4 mo- 2 years, often post viral, staph/strep, HSV, medications like penicillins, vaccinations b. Prev. thought to be similar to HSP but no IgA deposition, no GI or tenal effects c. On path C1q an IgM deposition d. Clincially- abrupt onset, annular, targetoid or nummular and pften purpuric plaques on FACE, EARS, extremities, often spares the trunk e. Child usually well, arthralgias in 56%, SIGNIFICANT edema f. Self resolving 1-3 weeks
470
18. What is one distinguishing feature of the urticaria in autoinflammatory syndromes
no itch
471
19. Name 3 congenital autoinflammatory syndromes
a. CAPS: Muckle-wells, NOMID, FCAPS  NLRP3 b. TNF-receptor associated periodic syndrome (TRAPS) = TNFR1 c. Hyperimmunoglublinemia D syndrome with. Periodic fevers. (HIDS)- mevalonate kinase
472
20. Name 2 acquired autoinflammatory syndromes
Stills Schnitzler
473
21. Name 4 differences between neutrophilic urticaria (variant of common urticaria) and NUD. *mostly path Neutrophilic urticaria shows:
i. Presence of dermal edema, neutrophils confined to the upper dermis, limited leukocytoclasia, similar numbers neuts:eos:monocytes ii. No association systemic dz vs. i. significant interstitial distribution of the neutrophilic infiltrate, along the collagen bundles ("en file indienne") and also in the deep part of the reticular dermis ii. significant leukocytoclasia is a constant feature in our experience. iii. Limited dermal edema iv. Eos/monocytes usually sparse v. Systemic disease
474
What is neutrophilic urticarial dermatosis? name 3 assoc. diseases
a. Variant of urticaria with neutrophilic infiltrate, LCV but no fibrin, minimdal dermal edema b. Painful > itchy c. Strongly associated with Stills, SLE, Schnitzlers
475
23. What is the 2 main cutaneous finding in Schnitzler
Neutrophilic urticarial dermatosis U
476
24. What are the diagnostic criteria for Schnitzlers
IgM monoclonal gammopathy urticarial lesions <24 hrs + 2 of following: 1. Bone pain-distal femur, prox tib 2. Abnormal bone scintirgraphy 3. Arthralgia, arthritis 4.LAD 5. HPS 6. ESR 7. Leukocytosis 8. Fever
477
25. What canSchnitzler progress to? What is the % progression to these diseases in Schnitlzers
i. 15% progress to Waldenstroms, Others include IgM myeloma, marginal b cell lymphoma, lymphoblastic lymphoma over 10-20 yrs
478
26. What is the most common trigger EM?
a. HSV-1 >2, precede out break by 1 week ish in 80% b. Also reported from covid
479
27. What is the clinical presentation EM
a. Start out monomorphic edematous papules on extremities, nay show actinic predilection and Koebner b. Develop into targetoir with dark/dusky ventre/ulcer, pale ring, then red inflammatory exterior c. Mild fever, mild mucosal sometimes,
480
28. Compare and contrast PEP and pemphigoid/gestationis
a. PEP: i. 3rd trimester more than pemphigoid is ii. Can flare at delivery but usually not after iii. Assoc w/ rapid weight gain, multiple gestations iv. Spares umbilicus v. May blister vi. Involves striae vii. No risk to baby for prematurity viii. Baby won’t get lesions ix. Does NOT rend to recur with future pregnancies, occurs at 1st pregnancy x. No association w/ thyroid ab’s xi. No HLA association xii. Path shows eosinophils and eos spong, negative DIF xiii. Responds to prednisone b. Pemphigoid gestationis i. 2nd trimester > 3rd ii. Can flare at delivery and often post partum iii. Involves umbilicus, no striae iv. Can occur with choriocarcinoma v. Recurs in subsequent pregnancies (or menses, OCP) vi. Risk prematurity vii. Baby can develop blisters viii. HLA DR4 association, Thyroid ab association ix. Path shows eos, eos spong, IgG1 and C3 deposition along BMZ x. Responds to pred
481
29. What is the UV thought to trigger PMLE
UVB
482
31. Where does PMLE occur? How long does it take for onset and offset?
a. V-neck, facial in kids b. Minutes, 7-10 days to improve c. Variants: i. Papular, papular-vesicular, plaque, urticarial, hemorrhagic, eczematous, vesiculobullous, EM like, prurigo like,
483
32. What is Wells syndrome
a. Eosinophilic cellulitis b. Granulomatous dermatitis with eosinophilia
484
33. What are the 3 stages Wells
a. Early- striking dermal edema with eos sup and deep b. Middle – eos, flame figures (eosinophilic MBP on collagen bundles), histiocytes c. Late – histiocytes and giant cells around flame figures, lesions gray-blue hue as resolve
485
Name 7 associations w/ Wells
a. Arthropod bite b. EGPA c. Hematological syndromes – polycythemia, lymphoma, leuk d. Hypereosinophilic syndromes e. Infection- dermatophyte, HSV, bacterial f. Drugs g. Solid tumors
486
35. Name 7 clinical variants of wells
a. Classic plaque- children b. Anular granuloma- adults c. Urticaria-like d. Pap-vesicular e. Pap-nodular f. Bullous g. FDE like
487
36. What is the DDX for flame figures:
I HATE BPH a. Insect bite b. Herpes/pemphigoid gestationois c. Atopic dermatitis d. Tinea pedis e. Eosinophilic cellulitis, EGPA Bulloous pemphgioi f. Psuedolymphoma g. Hypereosinophilic syndrome
488
What is eosinophilc annular erythema? How does it compare to Wells
a. Annular erythema with tissue eosinophilia without granulomatous inflammation or flame figures b. Often lethargy, fatigue c. Tx colchicine, sulfasalazine, plaquenil Both can have peripheral Eos
489
Name the different associations of Sweets
a. Drugs - GO SHLAM b. Post infectious- viral URTI, strep, yersinia c. Post vaccination d. Malignancy- AML, MDS, solid organ tumors, e. Inflammatory: IBD > RA, lupus, behcets f. IDIOPATHIC 70%
490
39. What are the associations with palisaded neutrophilic and granulomatous dermatitis / IGND
VIMID a. Vasculitis-ANCAs, takayasaus, EED, cryo b. Infection- subacute IE, hepatitis, strep c. Malignancy- lymphoma d. Inflammatory: IBD, RA, SLE, PMR, scleroderma , stills, sarcoid Drugs *RA and SLE
491
40. Name the 2 things on the ddx for PNGD/INGD
EED Granulomatous drug re
492
41. Name the 5 most common drugs to cause serum sickness like rxn
a. Cefaclor b. Minocycline c. Bupropion d. Infliximab e. Rituximab B-CRIM
493
Name 5 drugs associated with IGND
Hypertension-CCBs*, BBs, ACE inh Diuretics- Furosemide Statins* TNFa inh * Antihistamines Thalidomide Anakinra HD STAAT
494
Name 4 subtypes of miliaria
crystalina rubra pustulosa profunda
495
44. What is urticarial dermatitis
a. Hypersensitivity syndrome, dermal lympho-eosinophilic infiltrate with minimal epidermal change b. Very common c. Reaction drugs, supplements,food, id reactions Urticarial + dermatitic morphology in same plaque or
496
45. What is neutrophilic eccrine hidradenitis
a. Neutrophilic infiltration of eccrine glands b. Often painful papules plaques and nodules +- fever c. Associated w/ AML/CML, cytrabine, cetuximab, imatinib, antrhacyclin MC situation is acute myelogenous leukemia patients receiving chemotherapy, most commonly with cytarabine.
497
What is major and minor criteria of papuloerythroderma of ofuji? How does it present?
Urticarial to dermatitic polygonal flat topped papules and plaques that spares the folds, often erythrodermic and sometimes flat topped papules, most common in Japan Major -Erythrodermic eruption of flat topped coalescent red brown papules with cobblestone appearance -Spares skin folds -Pruritus -Pathological picture excludes lymphoma and other skin diseases -Absence of triggering factors like neoplasms, infections, drugs Minor -> 55 -Male -Peripheral or tissue eos -Elevated IgE -Peripheral lymphopenia
498
1. Name 2 Large vessel vasculitidies and 2 medium vessel vasculitides
Large: GCA, takayasaus Medium: PAN, KD
499
2.Describe the chapel hill vasculitis approach, highlight which are immune complex mediated
Large/medium as above b. Small vessel i. IgA/HSP ii. Urticarial vasculitis Hypocomplementemic/AntiC1Q iii. Cryoglobulinemic iv. EGPA v. GPA vi. MPA vii. Anti-GBM Variable vessel i. Behcets, cogans Vasculitis associated w/ systemic disease i. SLE, sarcoid, RA, Sjogrens Vasculitis w/ probable etiology i. Drugs, infections, sepsis, AI Cutaneous SOV i. IgG/IgM ii. Nodular vasculitis/ Erythema induratum of bazin iii. EED iv. Granuloma faciale v. Hypergammaglobulinemic macular vasculitis vi. Normoclomplementic urticarial vasculitis
500
Name 6 cutaneous features of PAN
a. Livedo reticularis or racemosa b. Subcutaneous nodules c. Punched out ulcers d. Hemorrhagic macules e. Retiform purpura f. Erythema
501
5. What are 3 systemic features that can be seen in cutaneous PAN
a. Fever b. Arthralgia c. Peripheral neuropathy in that limb
502
6. What genetic syndrome can you see PAN or cPAN-like features
ADA2 deficiency or :dada2
503
7. Name 4 underlying causes/triggers for PAN
a. Infections-HBV*, HCV* -Strep, Parvo, HIV with cutaneous PAN b. AI disease – IBD, SLE, familial med fever c. Malignancy – Hairy cell leukemia* d. Drugs (MTS) -Minocycline, tnf alpha, sulfasalazine For cutaneos PAN most commonly is chonric infections, AI CTD also often cutaneous
504
When might a PAN have a + P-ANCA
drug induced
505
8. What is KD diagnostic criteria
a. Fever 5 days + 4/5 of: i. Conjuctivitis (non purulent, bilat) ii. Rash-polymorphous iii. Adenopathy >1.5 cm iv. Strawberry tongue and mucosal changes (fissured lips) v. Hands and feet- palmar erythema/edema and desquamation
506
What are the 3 phases of EGPA? Name 3 cutaneous manifestations EGPA? What ANCA is most associated with EGPA and how often is it + and what does it mean? What systems are involved?
i. Adult onset asthma, allergic rhinitis, nasal polyps ii. Eosinophilic phase- pneumonia, GI, eosinophilia iii. Systemic vasculitis with granulomatous inflammation- cardiac, neurologic b. Skin findings i. Palpable purpura ii. Nodules iii. Ulcers iv. Urticaria early on c. ANCA? i. P-ANCA (MPO) in 40% ii. + indicates more neuropathy, - more cardiac d. Systems i. , cardiac, GI, upper and lower airway, neuropathy ii. NO RENAL
507
10. What are the 3 main systems involved in GPA? Name 5 cutaneous findings? How often cutaneous findigns? How often/what ANCA?
a. Upper and lower airway, renal b. Cutaneous in 46%-66% c. Skin i. Palpable purpura ii. Ulcerations resembling PG iii. Oral/nasal ulceration iv. Strawberry gums – gingival hyperplasia with friable tissue v. Papulonecrotic lesions vi. Subcut nodules d. C-anca/PR3 in 80%
508
11. How does MPA present? Who gets it? Name 3 lab findings?
a. Prodrome constitutional then explosive into pulm hemorrhage + renal RF+ often, P-ANCA/MPO in 90%,
509
12. What type of cryoglobulinemia is vasculopathic?
a. Type I- Monoclonal IgM, causes ulcers and infarcts
510
Describe the differences between Type I and Type II/III cryoglobulenemia
Mixed MONOCLONAL IgM (type I) OR POLYCLONAL (type II) IMMUNOGLOBULINS bind to polyclonal IgG (Fc portion) Type 1: Caused by monoclonal IgM>IgG (mono) Type 2: Monoclonal IgM or IgG against polyclonal IgG (mono-poly) Type 3: Polyclonal IgM against polyclonal IgG
511
14. What is cryoglobulinemia assoc with most often
HCV
512
15. Name the systemic findings of cryoglobulinemia? Name the cutaneous findings
a. SKIN, NERVES, KIDNEYS b. Skin-palpable purpura 90%, rarely erythematous papules, ecchymoses, and dermal nodules; i. rarely, urticaria, livedo reticularis, necrosis, ulcerations, and bullae are observed c. Systemic-peripheral neuropathy, GN
513
what is a RF
a. Antibodies directed against the Fc portion of IgG
514
17. Compare and contrast HSP in children and adults
a. KIDS- URTI MC cause vs. Adults-drugs and malignancy b. Renal involvement more common in adults (8-70%) > kids (2-15%) c. Abdo pain in kids vs. diarrhea in adults
515
18. What specifically is the immunoglobulin in HSP? Why is it different
IgA1-deficient in galactose in hinge region, makes it precipitate more easily
516
19. What systemic features can be seen in HSP?
a. Abdo pain b. Orchitis c. Arthralgias and arthritis d. GI- abdo pain, diarrhea, intussuception e. Peripheral edema f. GN
517
What are the 3 variants of hypocomplementic UV
a. Skin only b. As part of AI disease (Sjogrens, RA, SLE) c. Pulmonary, renal, eyes and arthritis
518
21. What is the diagnostic criteria for behcets (name both)
a. Need 4 points i. Oral apthae- 2 ii. Genital apthae-2 iii. Ocular involvement (uveitis) - 2 iv. Skin manifestations 1 v. CNS 1 vi. Vascular1 vii. Pathergy 1 point Vs. 3 of following: Skin-EN like, necrotic folliculitis, acneiform Oral apthae-3x/yr Genital ulcers Neuro Ocular- uveitis, vasvulitis retina Vascular -venous thrombosis, arterial thrombosis or aneurysms
519
22. What are the non ulcer skin mainfestations of behcets
a. Acneiform rash b. Necrotic folliculitis c. EN-like lesions  v speific d. EN
520
23. What is the MC SOV?
a. IgG/IgM vasculitis b. Often triggered by infection
521
24. What is the demographic for nodular vasculitis/erythema induratum? What is it
a. Lobular panniculitis w/ vasculitis b. Nodular lesions Backs of legs, women, 30-60, venous stasis, obese
522
25. What is erythema induratum of bazin
a. Lobular panniculitis with vasculitis b. Indicative of active Tb
523
26. How does EED present? What is it associated with
-Red–violet to red–brown papules, plaques, and nodules that favor extensor surfaces (elbows, dorsal hands, knees, ankles) -Nodular lesions, palmoplantar, that progress to form bulky masses --> consider HIV infection *High association IgA monoclonal gammopathy, AI CTD b. Associated with infection (strep, HIV, HBV, syphilis, tb), monoclonal gammopathy (esp IgA) and other heme malignancy, AI disease (GPA, RA, IBD, celiac, polychondritis)
524
What does granuloma faciale show on path? Who gets it ? triggers?
a. Mixed vasculitis with neutrophils, eosinophils, lymphocytes, plasma cells b. Grenz zone c. Perivascular infiltrate, +- vasculitis, +- IgG on DIF Telangiectasias Who gets it? Males, older/middle age Sun exposure
525
28. What is hypergammaglobulinemic macular vasculitis
( golfers/exercise induced vasculitis may also be a form of this) c. Macular purpura lower extremities, monoclonal or more often polyclonal gammoapathy , often associated w/ Sjogrens IgG e. Labs: ESR, poly/monoclonal gammopathy, Non igM RF
526
30. What is diagnostic criteria for HUVS (hypocomplement)
a. two major criteria : (1) urticaria for 6 months and (2) hypocomplementemia + b. two or more minor criteria: (1) vasculitis on skin biopsy; (2) arthralgia or arthritis; (3) uveitis or episcleritis; (4) glomerulonephritis; (5) recurrent abdominal pain; or (6) positive C1q precipitin test with a low C1q level.
527
What is the workup/ddx for LCV?
Viral (hep B/C) i. Hep B/C, HIV serology Autoimmune i. ANA, RF, ENA, UA, ANCAs, Strep, Staph, hSp, Skin bx w/DIF i. Throat swab, SPEP Cryoglobulinemia, cryofibrinogens i. Cryoglobulins, cryofibrinogins, creatinine UV Hypocomplementemic, UC/IBD i. C3/C4 ii. +- colonoscopy if indicated Lymphoproliferative-hairy cell i. CBC, LDH, SPEP, smear Infectious-endocarditis, meningococcemis, gonoccoal arthritis, RMSF i. Blood cultures, ii. Gram stain and culture bx Thiazide diuretics + other drugs, traumatic Immune complex, immune sera Septra and other abx
528
3 phases of raynauds
white blue red, sometimes no blue
529
8 RF for primary raynauds
Thin Smoking cold climate cv disease manual occupation drugs older age female gender, family history of PRP, migraine, smoking, CVD , oestrogren replacement therapy
530
10 features of primary raynauds vs. contrast to secondary: Sex Age Frequency Precipitants Ischemic injury Capillaroscopy Other vasomaotor instability ANA + In vivo PLT activation Fam hx
Sex: Strong female predominance vs. only 4:1 M:F Age: puberty vs. >25 <5 vs. >5 attacks daily Precipitants: cold or stres vs. cold Ischemic injury and abnormal capillaroscopy absnet in primary Other vasomotor phenomenon present in both All ab's absent in primary ANA + in almost all secondary: i. CREST -ACA+ ii. Diffuse systemic SCL70 iii. SLE sm+ iv. Sjogrens SSA/SSB Others: Fam hx strong in primary Progressivr disease in secondary
531
35. Percent of people healthy with ANA 1:80, 1:160, 1:320
13%, 5%, 3.3%
532
Name 3 general causes for seconday raynauds and give 5 examples of each
a. Vasculopathy i. CTD (scleroderma>SLE>sjogrens), traumatic (frost bite, vibration), drug (chemo), buergers, vasculitis, thpracic outlet, brachiocephalic trunk dz e.g. takayasaus, athero b. Vasospastic i. Drugs (ergots, sympathetic agonists), endocrine (pheo, carcinoid, hypothyroid), neuro (nerve root entrapment) c. Coagulation i. Cryoglob, cryofirin, paraproteins, macroglobulins, cold agglutinin, PCV, thromboembolism
533
37. Name 3 1st, second and third line tx for raynauds
a. 1st: nifedipine/amlodipine, topical nitro, ASA, dipyramidole b. 2nd: PDE-5 (sildenafil), ARB, pentoxifylline c. 3rd: Endothelin receptor antagnoists (Bosentan), Prostaglandin E1 infusion, biofeedback, LMWH
534
5 general tx for digital ulcers
a. Pain control b. Soak BID in ½ strength H202 c. Abx ointment and occlusive dressing d. Hydrocolloids/gels e. Max CCB therapy
535
Name the 6 types of scleroderma
a. Morphea-localized scleroderma b. Prescleroderma c. Systemic sclerosis-limited d. Systemic sclerosis-diffuse e. Sine scleroderma f. MCTD
536
40. Limited systemic sclerosis: which ab most associated? What systemic finding do they often get? What nailfold change?
a. Anti centromere b. Pulm htn c. Tortuous capillaries, but no dropout
537
41. In systemic sclerosis diffuse, what nailfold changes are seen? Name 2 other cutaneous features? Name 5 internal organ findings and the most common Ab
a. Ptergyium unguis inversum, confetti leukoderma b. Tortious capillaries with dropout c. Internal i. Cardiac, GI, ILD, oliguric renal failure, tendon friction rubs, polyarticular synovitis d. Ab: SCL-70/topoisomerase in 30%
538
42. Name 4 new treatments for scleroderma
a. Tocilizumab (anti-IL-6) b. Rituximab +- MMF c. PDE-5 inhibitors, endothelin receptor antagonists, prostacyclin analogues d. Antifibrotics: Nintendanib
539
43. List the 12 SLICC criteria (need 4 or more)
a. ACLE or SCLE b. Chronic LE c. Non scarring alopecia d. Oral ulcers e. Serositis f. Arthritis/arthralgias g. Lupus cerebritis/neuro h. Lupus nephritis i. Hemolytic anemia j. Leukopenia or lymphopenia k. Thrombocytopenia l. Immunologic: ANA, dsDNA, smith, low complement, APLAS, DAT)
540
44. What are 4 changes on path that help distinguish lupus specific skin changes and 2 more in general
a. Increased mucin b. BMZ thickening c. Perivascular and periadnexal lymphocytic sup and deep infiltrate d. Interface dermatitis e. General: hyperkeratosis, follicular plugging , epidermal atrophy
541
45. Name the Lupus specific lesions
ACLE-malar and generalized SCLE- psoarisiform or annular, TEN-like CCLE- DLE, panniculitis, profundus, tumid, chilblains
542
46. Name 10 non lupus specific lesions
Vasculitis APLS changes/vasculopathy( nodules, ulcers, atrophie blanche) livedo RP non-scarring alopecia, oral ulcers, periungual telangiectasias, erythromelalgia, bullous lupus, rheumatoid nodukes, dermatofibromas, lupus mucinosis, sclerodactylyl, acanthosis nigricans, anetoderma, CSU, alopecia areata
543
47. Name 7 drugs that can cause SLE, what ab’s associated, and 1 clinical difference, and 1 drug that’s different
a. Drugs “HIP MC” i. Hydralazine #1 ii. Isoniazid #2/3, interferon iii. Procainamide #2/3, phenytoin , penicillamine (unmasks dz) iv. Minocycline (anti MPO), Methyldopa v. Chlorpromazine vi. TNF-inhibitors b. Ab: anti-histone in 95% c. No skin involvement usually d. TNF dsDNA +, skin present often, more CNS and more renal
544
48. What are MC Abs in SCLE? Name 5 causative drugs . What % SCLE is drug induced
a. Ab: SSA 90%, SSB40%, ANA 70% b. Drug induced also usually SSA/SSB + in c. 50% drug induced d. Drugs: THIN DRAGS i. Terbinafine, TNF alpha, taxanes ii. HCTZ #1 iii. Interferon iv. NSAIDS v. Diliazem/CCBs vi. Ranidtinde/PPI/PUVA vii. Acei/antiepileptics viii. Griseofulvin ix. Spironoloactone, sulfonylureas
545
49. Name 4 RF for SLE in SCLE, and what % of SCLE pts have SLE?
a. ANA+ b. dsDNA c. Papsquam>annular variant d. Leukopenia
546
50. Name 4 internal organ systems present in neonatal lupus? What Ab is in them? Which RO subtype is more associated with lupus? Sjogrens?
a. Organs i. Heart block ii. Transamninitis/cholestasis/hepatic failure iii. Cytopenias iv. Macrocephaly v. *annular skin lesions b. SSA >SSB c. SSA 52 KD w/ SJOGRENS  may have higher risk heart block d. SSA 60 KD w/ LUPUS
547
51. What is risk SLE with DLE localized, generalized, chilblains and profundus
a. Localized- 20% b. Generalized- 50-70% c. Chillblains 20% d. Profundus 50%
548
52. Name 6 tx for cutaneous lupus
a. SPF 50 + b. TCS, TCI, crisaborole, topical retinoids, topical dapsone c. ILK d. Anti-malarials e. Acitretin if hyperkeratotic f. Immune suprresants: AZA, MMF, mtx, apremilast, csa, leflunomide. g. Biologics: ustekunumab, ritux, MC: Rituximab, Belimumab
549
53. What is dosing for HCQ, chloroquine and quinacrine
a. HCQ 5 mg/kg actual BW b. Chloroquine 2.3 mg /kg actual BW c. Quinacrine 100-200 mg po daily
550
54. Name 8 RF for ocular toxicity in HCQ or chloroquine
a. Dosing > 5 m/kg or 2.3 for chloroquine b. > drug 5 yrs c. Renal disease d. Hepatic disese e. Macular degeneration, retinal dystrophy f. > 60 g. Obese h. Tamoxifen
551
Name treatments for: livedoid vasculopathy
a. Compression b. clopidogrel c. Topical steroids or ILK d. Pentoxyfilline e. Nicotinamide
552
56. What is seen on path for bullous Lupus
Linear or granular IgG and C3, +- IgM and IgA at DEJ Floor on SSS Lesions and perilesional
553
57. Top 3 tx bullous SLE
dapsone colchicine sulfasalazine
554
Name 9 features of JDM more prevalent in juvenile than adult
a. Vasculitis b. Vasculopathy c. Calcinosis -superficial nodules and plaques -Calcinosis circumscripta -calcinosis universalis -exoskeleton pattern d. Dystrophic calcification e. Gingival telangiectasia f. Arthritis g. Muscle atrophy and contracture h. Retinopathy i. Large bowel infarction/perf secondary to vasculitis
555
59. Name top 5 cancers seen in adult dM? what is the RR in the first year
a. Ovarian, breast, lung, gastric, nasopharyngeal b. 26 x risk in 1st year
556
What is the workup required for DM in adults cancer screening wise
a. CT CAP b. Colonoscopy c. TVUS d. Mammogram e. CBC, LDH f. Tumor markers
557
61. Name the pathognomonic findings of DM, characteristic findings, and suggestive findings
Pathognomonic: Gottrons papules, gottrons sign Characteristics i. Shawl sign, Holster sign ii. Heliotrope iii. Periungual erythema +- cuticular dystrophy iv. Mechanics hands c. Suggestive i. Calcinosis cutis ii. Seb-like dermatitis iii. Flagellate erythema iv. Lymphocytic lobular panniculitis v. Poikiloderma vasculare atrophicans
558
62. Name 5 features anti synthethase syndrome
a. Mechanics hands b. ILD c. RP d. Arthritis e. Fever
559
63. Name 4 DM treatments pre malignancy and once malignancy ruled out
Pre-malig i. Prednisone ii. HCQ iii. IVIG iv. MTX Malig rules out i. AZA, MMF, Csa, Ritux * esp if vasculitis
560
64. How often to repeat malignancy workup
a. At 1-2 years b. If no malignancy in first 2 years, decreased risk, at baseline at 5 yrs
561
65. Name 10 organs sarcoid can affect and 1 manifestation of each
a. Skin- specific vs. reactive b. Constitutional-fever, weight loss, sweats c. Lung (90%)- SOB, cough d. Liver- increase ALP e. Eye -uveitis f. Lymph nodes-asymptomatic g. Spleen h. Heart- heart block, cardiomyopathy i. Hematologic – lymphopenia, eosinophilia j. Hypercalcemia- kidney stones k. Brain – CN palsies, seizures l. Joints- ankles, knees, wrists, cysts
562
66. What type of granulomas are sarcoid?
non caseating naked often
563
67. What % sarcoid in skin will go on to have systemic involvement
60-80%
564
Approach to sarcoid in the skin
a. Reactive/non specific: no granulomas, usually acute and self limited sarcoid i. E.g. EN, pruritus, EM, nummular dermatitis, calcinosis cutis, clubbing, prurigo Specific: Granulomas present Acute 1. Maculopapular 2. Nodular Chronic: AAAPPI 1. Lupus pernio 2. Angiolupoid 3. Plaque 4. Annular 5. Alopecic 6. Icthyotic No association with acute or chronic 1. Darier roussy nodules 2. Scar sarcoid 3. Tattoo sarcoid 4. Drug induced 5. Follicular 6. Ulcerative 7. Psoriasiform 8. Hypopigmented 9. Rarer-erythrodermic, lichenoid, perfortating, hypertrophic, imbilicated
565
iv. What are the 4 major organs to screen for in sarcoid
Heart Lungs Eyes Brain
566
What is the workup for sarcoid
i. H+P including neuro, eyes, heart, lungs ii. Biopsy for H+E and culture iii. Labs: CBC, LE, Ca, ACE level, CD4:CD8 level, serum immunoglobilins iv. EKG and TTE +- cardiac MRI v. CXR and PFTs vi. TBST viii. Refer: Optho, resp, IM, cardio
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69. What is the prognosiss for sarcoid
a. 60% spont. Resolution b. 30% progressive/chronic
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70. Name 5 tx for EN lesions in sarcoid (reactive)
a. Bed rst b. NSAIDS c. ILK d. Pred e. SSKI
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71. Name 8 tx options for specific lesions of sarcoid
a. TCS b. ILK c. Anti malarials d. Tetracyclines e. Allopurinol f. Pentoxyfilline g. Pred h. MTX i. AZA
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What are the diagnostic criteria for Sjogrens
Inclusion: dry eyes or mouth x 3 months, not explained by something else Need 4 points or more i. Salivary gland bx showing focal lymphocytic sialdenitis and focus score of 1 FS/ 4 mm2 or greater (3) ii. SSA + (3) iii. Schirmer test <5 mm/5min in one eye (1) iv. Ocular staining score (>5) in one eye (1) v. Unstimlated whole saliva flow rate <0.1 ml/min Exclusion criteria: i. Hep C, AIDS, head/neck radiation, sarcoid, amyloid, GVHD, IgG4 related disease
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73. Name the 3 most common glandular manifestations of sjogrens
a. Xerostomia b. Keratoconjuctivitis sicca c. Vaginal tract dryness
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74. Name 8 systemic manifestations Sjogren
a. Fatigue b. MSK- arthralgia, non erosive arthritis c. Neuro- small fibro neuropathy, peripheral neuropathy, transverse myelitis, , dementia, psych) d. Pulmonary- xerotrachea, ILD, lymphocytic pneumonitis e. Vascular- vasculitis f. GI- gastroparesis, g. Liver-PBC, AI hepatitis h. Nephro- tubuluinterstialn nephritis i. Heme- lymphopenia, B cell lymphoma, polyclonal gammopathy
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NAme the 12 cutaneous features Sjogrens
a. Xerosis "Vasculitis" b. LCV c. Waldenstroms benign hypergammaglobulinemic purpura d. UV e. Cryoglobulinemic vasculitis "Urticarial" f. Annular erythema of sjogrens g. Urticaria "AI-CTD" h. RP Neonatal lupus i. Livedo reticularis "Infiltrative" j. Marginal zone lymphoma k. Nodular amyloid

MyDermatology-Bolognia (31 decks)

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