General Dermatology Flashcards
1
Q
Which medications can induce a flare of pustular psoriasis (8)?
A
Beta-blockers Lithium IFN Antimalarials (plaquenil) TNF-inhibitors NSAIDs Terbinafine CCBs
2
Q
Erythema gyratum repens is associated with which malignancies (5)?
A
Lung = #1 Breast Cervical Bowel Bladder
3
Q
Name the 3 longitudinal sections of the hair follicle and an associated disease with each section.
A
- Infundibulum (surface of skin to sebaceous gland opening): LPP
- Isthmus (sebaceous gland opening to insertion of arrestor pili): DLE
- Inferior segment (the rest): alopecia areata
4
Q
What is the most common mutation in mucosal melanoma? In uveal melanoma?
A
Mucosal melanomas: KIT
Uveal melanomas and blue nevi: GNAQ
5
Q
Which cutaneous manifestations are more common with Ulcerative Colitis than Crohn’s? …more common in Crohn’s than UC?
A
UC > Crohns: erythema nodosum, pyoderma gangranosum, pyoderma vegetabs
Crohns > UC: oral disease, fissures, fistulae, metastatic disease to skin, PAN
6
Q
Verruciform xanthomas are seen in which conditions? What is the most common location?
A
CHILD syndrome, EBA, GVHD, lymphedema syndromes
Most common location = oral mucosa
7
Q
How do you calculate the dose of UVR a phototherapy patient should receive?
A
Irradiance (Watts, which is power/unit area) x time (seconds) = UV dose (Joules, which is energy/unit area)
8
Q
What to DSAP and Hyper IgD have in common? What are manifestations of Hyper IgD?
A
MVK mutation (melavonate kinase gene) - regulates calcium-induced keratinocyte differentiation and may protect from UVA-induced apoptosis
HIDS: periodic fevers (induced by vaccines, etc), nausea/vomiting/diarrhea, LAD, rash
9
Q
What are the mutations and distinguishing features of the following auto inflammatory conditions: Muckle-Wells, Familial Mediterranean Fever, TRAPS?
A
*All have periodic fevers!
Muckle-Wells: CIAS1 mutation (cryopyrin); AD; urticaria, deafness, renal amyloidosis (AA), abd pain, myalgia/arthralgia, conjunctivitis
– Tx = Anakinra, steroids
Familial Mediterranean Fever: MEFV mutation (pyrin); AR; polyserositis, erysipelas-like erythema of legs
– Tx = colchicine
TNF Receptor Associated Periodic Syndrome: TNFR1 mutation; AD; erythematous serpiginous patches/plaques on extrems, abd pain, arthralgia/myalgia
– Tx = TNF-alpha inhibitor, steroids
10
Q
Name the porphyrias that “HAVE” abdominal pain.
A
Hereditary coproporphyria
Acute Intermittent Porphyria
Variegate Porphyria
Erythropoietic Porphyria (also has cholelithiasis)
11
Q
Androgens:
- Most potent? How is it made?
- Where are androgen receptors that are relevant to acne?
- Which androgens are made in the adrenals?
A
- Dihydrotestosterone (DHT) = most potent.
- Testosterone is made in gonads and converted to DHT by 5-alpha-reductase.
- Dehydroepiandrosterone sulfate (DHEA-S) and 17-hydroxyprogesterone = weak androgens made in adrenals
Androgen receptors on sebaceous glands and outer root sheath of hair follicle
12
Q
How does estrogen help acne?
A
Decreases sebum production
Directly opposes effects of androgens on sebaceous glands
Inhibits production of androgens by gonads
13
Q
In psoriasis, which cytokine stimulates Th1? Th17? Which cytokine correlates with disease severity
A
IL-12 stimulates Th1
IL-23 stimulates Th17
IL-22, a Th17 cytokine, correlates with disease severity
14
Q
Which medications can cause drug-induced lichen planus (typically NO Wickam’s striae)?
A
Beta-blockers Captopril Penicillamine HCTZ Anti-malarials Quinidine NSAIDs Tetracyclines Gold Sulfonylureas Methyldopa Carbamazepine 5-FU
15
Q
Type of Amyloid deposited in these conditions: Macular, Lichen, Nodular, Secondary, Primary Systemic, Secondary Systemic, Dialysis-Associated, Familial, Senile Systemic
A
Macular -- AA Lichen -- AA Nodular -- AL Secondary -- AA (derived from liver) Primary Systemic -- AL Secondary Systemic -- AA (periodic fevers, RA, other inflammatory states) Dialysis - Beta-2 Microglobulin Familial -- ATTR (transthyretin) Senile -- ATTR
16
Q
Muckle Wells Syndrome
- Mutation
- Treatment options
A
Mutation: CIAS1 (cryopyrin)
Treatment:
- steroids
- Anakinra (IL-1 receptor antagonist)
- Canakinumab (mAb against IL-1)
- Rilonacept (IL-1 -TRAP fusion protein)
17
Q
Familial Mediterranean Fever
- Mutation and Inheritance
- Features
- Treatment
A
MEFV mutation (Pyrin)
AD inheritance
Features: hives, polyserositis, fever, erysipelas-like erythema on legs
Tx: Colchicine
18
Q
TNF Receptor Associated Periodic Syndrome (TRAPS)
- Mutation and Inheritance
- Features
- Treatment
A
TNFR1 mutation; AD
Features: fevers, red patches/plaques on extremities, and pain, arthralgias/myalgias
Tx: TNF-inhibitors, glucocorticoids
19
Q
Scleredema
- What are the 3 forms?
- Treatment?
A
I: infection-related – often strep; self-limited induration of cervicofacial area and proximal extremities
II: Gammopathy associated – IgG kappa; insidious onset
III: Diabetes-related – slow onset of induration on neck/back in obese men with IDDM; sharply demarcated normal skin; treating DM does NOT improve.
Tx: Phototherapy, cyclophosphamide, steroids, cyclosporine
20
Q
Erythropoietic Protoporphyria
- Mutation and inheritance
- Features
- Treatment
A
Ferrochetolase (AD)
EPP = “empty PP but gallbladder full of stones”
Features: photosensitivity with burning, scars, milia, gallstones, hepatic damage
Tx: light avoidance, beta-carotene
21
Q
Porphyria Cutanea Tarda
- Mutation and inheritance
- Features and Triggers
- Treatment
- AR form’s name and features
A
Uroporphyrinogen decarboxylase (AD or acquired)
- Features: bullae, erosions, milia, hypertrichosis, scleroderma-like changes
- Triggers: alcohol, HCV, estrogen, polychlorinated hydrocarbons, iron overload (hemochromatosis), HIV
- Tx: phlebotomy, plaquenil
- AR form = Hepatoerythropoietic Porphyria (features of PCT and congenital erythropoietic protoporphyria; Tx = photo protection)
22
Q
Variegate Porphyria
- Mutation and Inheritance
- Features
- Treatment
A
Ptoroporphyrinogen deaminase (AD)
- Features: combo of PCT and Acute Intermittent (neuro/psych findings, abdominal pain; AIP has no skin findings); plasma fluoresces at 626 nm
- Tx: during attacks glucose load, hematin infusion; avoid triggers (alcohol, drugs, stress, fasting, hormone change, infection)
23
Q
Familial Hyperlipidemia
– Mutation, lipid levels and characteristic skin findings of Type I, Type IIa, Type IIb, Type III, Type IV, Type V
A
Type I: LPL deficiency, elevated TG, Eruptive xanthomas and acute pancreatitis
Type IIa: LDL receptor defect (either LDLR or Apo-B mutated); elevated LDL; tendinous/tuberous xanthomas (interdigital webspace pathoglomonic); statins dont help b/c no LDLR
Type IIb: Familial combined; LDL receptor defect; elevated chol/TG; tendinous/tuberous xanthomas
Type III: ApoE protein defect (chylomicrons not cleared); elevated Chol and TG; Xanthoma striatum palmare pathognomonic; tuberous xanthomas
Type IV: Increased production of VLDL; elevated TG; eruptive xanthomas; assoc w/ DM, obesity, alcoholism
Type V: apolipoprotein C-II defect; increased TG/chol; Eruptive xanthomas, pancreatitis, DM
24
Q
What mediators are pre-formed in mast cells (5)?
A
histamine heparin tryptase chymase serotonin
25
Types of Hereditary Angioedema and what is dysfunctional.
Type I: Decreased C1-INH level (85% of cases)
Type II: normal C1-INH level but dysfunctional (15% of cases)
Type III: estrogen dependent; activating mutation in factor XII; normal C1-INH level (rare)
Labs for all show decreased C4 (type III has normal C4!), Decreased/Normal C1-INH, normal C1q
26
Expected lab values in hereditary vs acquired angioedema
Hereditary Angioedema: low C4, low/normal C1-INH, normal C1q
Acquired Angioedema: low C4, low/normal C1-INH, LOW C1q
*Acquired due to anti-C1 INH Abs OR is secondary to a lymphoproliferative process in which immune complexes are consuming C1q
If C4 normal, angioedema is drug-induced or idiopathic
27
Causes of Drug-Induced Pemphigus Vulgaris (many)
Thiol drugs (penicillamine (PF > PV), captropril, enalapril, lisinopril, prioxicam); pyrazolone derivatives (phenylbutazone, oxyphenylbutazone); antibiotics (penicillin derivatives, cephalosporin, rifampicin)
28
Causes of drug-induced pemphigus foliaceous
Penicillamine, nifedipine, ACEIs
29
Malignancies associated with paraneoplastic pemphigus, in order of frequency (6)
```
Non-Hodgkin's Lymphoma (40%)
Chronic lymphocytic leukemia (30%)
Castleman's (10%)
Thymoma (6%)
Waldenstrom macroglobulinemia (6%)
Sarcomas
```
30
Autoantigens implicated in paraneoplastic pemphigus
All plakins + BPAG1...
Desmoglein 3, periplakin, envoplakin, desmoplakin, BPAG1, plectin, alpha-2 macroglobulin-like 1 (A2ML1 - 170 kDa)
NOT BPAG2! Must have Abs to either Dsg1 or Dsg3 which initiates blister formation
31
causes of drug-induced Bullous Pemphigoid?
Furosemide, NSAIDs, phenactin, PCN-derivatives, gold, potassium iodide, captopril, enalapril, penicillamine, sulfasalazine
32
Autoantigens in cicatricial pemphigoid and implications...
```
BPAG2 (c-terminus): mucosa and skin
Beta4 subunit of a6b4-integrin: OCULAR
Laminin 332 (epiligrin): cancer risk (adenocarcinoma)! -- binds DERMAL side of SSS
Laminin 6
BP230
```
33
Paraneoplastic Pemphigus vs Cicatricial Pemphigus and the lip...what differentiates the two?
PNP involves vermilion lip; CP does NOT.
34
Gluten is found in...
It is NOT found in...
Found in: wheat, rye, barley
NOT: rice, oats, corn
35
What are Anti-Ro (SSA) and Anti-La (SSB) associated with in Lupus? In Sjogren's?
Lupus: mild SLE, photosensitivity, SCLE, neonatal LE/congenital heart block
Sjogrens: increased risk of systemic disease and lymphoma
36
Which antibody is most specific for Sjogrens?
Anti-alpha-fodrin (actin binding protein) is 93% specific
37
What are the following antibodies associated with in dermatomyositis? What is their target antigen?
- - Anti-155 kDa Ab
- - Anti-Jo1
- - Anti-SRP
- - Anti-Mi2
- - Anti-Ku
- - Anti-CADM
- - Anti-155 kDa Ab (uncharacterized): Amyopathic DM, cancer-associated DM
- - Anti-Jo1 (Hidtidyl tRNA synthetase): Antisynthetase syndrome with Raynayd's, mechanic hands, pulm fibrosis
- - Anti-SRP (signal recog protein): Cardiac disease, severe DM/PM, poor prognosis
- - Anti-Mi2 (nuclear helicase): good prognosis, classic skin findings
- - Anti-Ku (p70/80 protein): DM/PM overlap with SLE or scleroderma
- - Anti-CADM (?): skin ulcerations, poor prognosis, possible malignancy assoc
38
What % of SCLE patients have a positive ANA? Positive Anti-Ro (SSA)? What HLA types are associated with SCLE?
ANA 60-80%
Anti-Ro 60-90%
HLA types: HLA-B8 strongest, HLA-DR3, HLA-DRw52, HLA-DQ1
39
What are the diagnostic criteria for SLE?
Need 4 of 11: malar erythema, discoid LE, photosensitivity, oral ulcers, + ANA, Arthritis, Serositis, Hematologic disorder, Immunologic (sDNA, Sm), Neuro disorder, Nephropathy
40
What are the most common malignancies associated with dermatomyositis in men / women?
Men: Lung, GI cancer
Women: Ovarian, Breast
41
If a dermatomyositis patient has anti-MDA5 antibodies, what does this mean?
MDA5 = melanoma differentiation associated gene 5
Associated with cutaneous manifestations including ulcerations, interstitial lung disease, malignancy. Do more intense malignancy evaluation (CT c/a/p)
42
What are the features of Mixed Connective Tissue Disease? Characteristic, but not specific, antibody?
Must have -- Raynaud's, Dactylitis, Arthritis
Can Have -- fever, sclerodermoid/poikiloderma of trunk, esophageal dysmotility, pulm HTN
Ab: Anti-U1RNP
43
What differentiates Brunsting and Baker types of Dermatomyositis?
Brunsting: slow, progressive with weakness and calcinosis; steroid responsive
Baker is evil: vasculitis of muscle/GI tract; rapid onset severe weakness; steroid resistant --> Death
44
Which antibody is most specific for Sjogren's? Which other antibodies (2) are commonly found?
Anti-alpha-fodrin (antigen = actin binding protein) = 93% specific
Also see Anti-Ro, Anti-La
45
What malignancy are patients with Sjogren syndrome at risk for?
B-cell lymphomas, including MALT and non-Hodgkins
Increased risk of present with vasculitis.
46
Which cells are increased in nephrogenic systemic sclerosis?
CD34+ T-cells
47
Which conditions is relapsing polychondritis associated with (3)? HLA-association? Antibodies?
Myelodysplastic syndrome, Behcet's disease, MAGIC (Mouth And Genital ulcers with Inflamed Cartilage)
HLA-DR4
Antibodies to type II collagen (same in MAGIC)
48
Which medications can cause accelerated cutaneous nodulous in RA patients? Affected sites? Treatment?
MTX >>> etanercept, infliximab, leflunomide
* Rapid onset on hands/feet/ears and can also occur in lungs, heart, meninges
* Treatment: Colchicine, D-penicillamine
49
What is the differential diagnosis of panniculitis with neutrophils (7)?
pancreatic panniculitis, alpha-1-antitrypsin, Weber-Christian, infectious conditions, cold panniculitis (mixed), subcutaneous Sweets, early erythema nodosum (lymphs later)
50
What differentiates Pemphigoid Gestations from PUPPP?
PG: involves umbilicus, starts 2nd trimester - immediate postpartum, + IF and NC16A ELISA, fetal risk of prematurity and SGA; treat with systemic steroids. Maternal risk of Graves! Typically recurs with subsequent press/OCPs/menstruation.
PUPPP: starts late 1st trimester of 1st pregnancy; does not recur. SPARES umbilicus. No fetal risk. Tx topical steroids and antihistamines; resolves 7-10 days after delivery.
51
Which drugs are often culprit of drug-induced cutaneous small vessel vasculitis?
Common: NSAIDs, COX-2 inhibitors, leukotriene inhibitors, penicillins, quinolones, anti-TNF, G-CSF, hydralazine, propylthiouracil
Others: ACEIs, allopurinol, furosemide, quinine, macrolides, thiazides, sulfonylureas, TMP-SMX, vancomycin, IFN, beta-blockers, penicillamines, MTX, OCPs, cocaine with levamisole, retinoids, sirolimus, warfarin, SSRIs, contrast media
52
Diagnostic criteria for Henoch-Schonlein Purpura
Palpable Purpura + 1 of the following:
- - Arthritis / Arthralgia
- - Diffuse abdominal pain
- - Predominant IgA deposition on biopsy
- - Renal involvement
53
Three phases of Churg-Strauss (Eosinophilia with granulomatosis and polyangiitis)? Lab abnormalities?
Labs: elevated IgE and eos; p-ANCA positive (anti-myeloperoxidase)
1st Phase: allergic rhinitis, nasal polyps, asthma
2nd: peripheral eosinohpilia, resp infections, GI symptoms
3rd: systemic necrotizing vasculitis with granulomas
54
Common presenting signs of Granulomatosis with Polyangiitis (Wegeners)? Expected Lab?
Skin: strawberry gingiva, oral ulcers, painful nodules/ulcers that mimic PG
Other: nose/sinus/ear complaints typical because upper and lower respiratory tracts involved
C-ANCA positive in 80%
55
Which 5 factors predict increased risk of mortality in Polyarteritis Nodosa?
Creatinine > 1.58, Proteinuria > 1g/day, GI tract involvement (bleeding/perforation/infarction/pancreatitis), CNS involvement, Cardiac involvement
*Whether HBV alters mortality is up for debate (KW and Jean Bologna are debating...). Orchitis is more common if HBV+.
56
Name the lupus anticoagulants (3)
Anti-beta2-glycoprotein
Lupus anticoagulant
Anti-cardiolipin
57
What is Degos Disease?
Malignant Atrophic Papulosis
* LETHAL small vessel angiopathy of skin, GI tract and CNS
- - crops of small erythematous papules --> porcelain white scars
- - Ischemic infarcts cause death (GI > CNS)
* Patients often present with stroke, GI bleed or acute abdomen
58
Diagnostic criteria for Behcet's disease.
Oral ulcers 3x/12 months + at least 2 of the following:
- - recurrent genital ulcers
- - positive pathergy test
- - ocular (uveitis, retinal vasculitis)
- - skin findings (ulcerations that can be acneniform, papulopustular, EN-like, pseudofolliculitis)
59
Diagnostic criteria for Sweets syndrome.
Abrupt onset of typical eruption with histopath findings + 2 of the following:
- - Preceded by associated infxn, vaccination, drug, malignancy, inflammatory condition, pregnancy
- - fever and constitutional symptoms
- - leukocytosis
- - excellent response to systemic corticosteroids
60
Drugs associated with Sweet's syndrome
G-CSF, ATRA, hydralazine, carbamazepine, levonorgestrel/ethinyl estradiol, TMP-SMX, minocycline
61
Systemic diseases associated with pyoderma gangrenous
IBD (UC > Crohn's), arthritis, hematologic disorders (IgA monoclonal gammopathy; multiple myeloma), HCV
62
What are patients with Vogt-Koyanagi-Harads syndrome at risk for? Skin findings?
Blindness from bilateral granulomatous uveitis (systemic steroids can prevent).
Skin finding: vitiligo
Other finding: aseptic meningitis
63
Thiamin (vitamin B1) Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
Berberi
- - Skin: glossitis
- - peripheral neuropathy (dry Beriberi), congestive heart failure (wet Beriberi), Korsakoff's syndrome, Wernicke's encephalopathy
- - Function: coenzyme in carbohydrate metabolism
- - Who: polished rice diet, alcoholics, Gi disease, hyperthyroid, diarrhea, malnutrition
64
Riboflavin (vitamin B2) Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
Oral-Ocular-Genital Syndrome
- - Skin: angular cheilitis, magenta colored tongue, seb derm, genital dermatitis, photophobia
- - Conjunctivitis
- - Function: energy prod, fatty acid / amino acid synthesis
- - Who: Celiac disease, alcoholics, malnutrition
65
Niacin (vitamin B3) Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it? Associated meds?
Pellagra (4Ds -- dermatitis, diarrhea, dementia, death)
- - Skin: photosensitive eruption, Casal's necklace, angular cheilitis, perianal dermatitis
- - Other: stomatitis, diarrhea, disorientation, coma
- - Function: red-ox reactions; tryptophan precursor amino acid
- - Who: corn diet, alcohol, isoniazid use, colitis, diarrhea, carcinoid syndrome; azathioprine and 5-FU can induce as well.
66
Pyridoxine (vitamin B6) deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: seb derm, atrophic glossitis, angular cheilitis, conjunctivitis
- - Other: dementia, peripheral neuropaths
- - Function: amino acid and fatty acid synthesis
- - Who: cirrhosis, uremia, INH therapy, malnutrition
67
Cobalamin (vitamin B12) deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: hyperpigmentation, glossitis, premature gray hair
- - Other: megaloblastic anemia, paresthesias, peripheral neuropaths, irritability
- - Function: DNA synthesis, neuro function
- - Who: GI absorption issues, vegetarians
68
Folic Acid Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: patchy diffuse hyperpigmentation, cheilitis, glossitis
- - Other: megaloblastic anemia (similar to B12 but without neuro findings)
- - Function: DNA synthesis
- - Who: elderly, MTX treatment, malnutrition
69
Biotin Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: similar to zinc def...periorificial eruption, conjunctivitis, alopecia; most common nutritional deficiency to be secondarily infected with CANDIDA
- - Other: depression, paresthesias, seizures
- - Function: essential cofactor for carboxylases
- - Who: acquired / inherited; ingesting raw egg whites, short gut and malabsorption
70
Vitamin A Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
Phrynoderma
- - Skin: keratotic follicular papules (KP-like), xerosis, keratomalacia
- - Other: night blindness, Bitot's spots (white spots on conjunctiva), bone changes
- - Function: normal keratinization of epithelial structures
- - Lab: check serum retinol to evaluate
71
Vitamin C Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
Scurvy
- - Skin: follicular hyperkeratosis, corkscrew hairs, perifollicular hemorrhage, petechiae, epistaxis, gingivitis, slow wound healing,
- - Other: IM and intraarticular hemorrhage --> pseudo paralysis due to pain
- - Function: collagen/ground substance formation
- - Who: alcoholics, fad diets, malnutrition
72
Vitamin D Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: alopecia
- - Others: rickets in kids, osteomalacia in adults
- - Function: regulates calcium-phosphate metabolism
73
Vitamin K Deficiency
- - Skin findings
- - Other findings
- - Function
- - Who gets it
- - Skin: purpura, ecchymosis, hemorrhage
- - Other: prolonged PT
- - Function: synthesizes coagulation factors II, VII, IX, X and protein C and S
74
Describe Vitamin D metabolism and skin's role
dehydrocholesterol absorbs UVB --> vitamin D3 (cholecalciferol) --> hydroxylated by liver to 25-OH-D3 --> hydroxylated by kidney to 1,25-OH2-D3 (calcitriol, the active form)
75
What is Bazex Sign? What does it signify?
Acrokeratosis neoplastica
- - psoriasiform plaques on palms, soles, nose, helices; NAILS INVOLVED! Can look eczematous
- - Upper aerodigestive tract SCC
76
Carcinoid Syndrome
- - Skin findings
- - Lab findings
- - Where is tumor?
- - Medical Tx
- - Skin: head/neck flushing, pellagra-like dermatitis (photosensitive, Casal's necklace, perianal dermatitis, etc), erythema, hyperhidrosis
- - Labs: elevated 5-HIAA in urine (serotonin metabolite)
- - Mid-gut tumors with liver and LN mets; #1 site is appendix but Ileum is most common location to cause Carcinoid syndrome
- - Tx: somatostatin, methylsergide, cyproheptadine, beta-blockers
77
Excess growth of lanugo hair is associated with which tumors?
Hypertrichosis lanuginosa acquisita
| -- Lung, Colon, Breast
78
Thick, velvety palms are associated with...
Tripe Palms
| -- lung cancer (if only palms); gastric cancer if palms + acanthosis nigricans
79
What is AESOP?
"Adenopathy and Extensive Skin patch Overlying Plasmacytoma"
-- enlarging violaceous patch with local andenopathy, usually around osseous structures on thorax
80
What can hyperkeratotic follicular papillose on the nose be a sign of?
Multiple myeloma
81
What is POEMS?
- - Skin findings?
- - Cancers (3)?
"Polyneuropathy, Organomegaly, Endocrinopathies, M-protein, Skin changes"
- - Skin: glomeruloid hemangioma (pathognomonic but rarely seen), other angiomas, hyper pigmentation, hypertrichosifs, hyperhidrosis, sclerodermatous changes, digital clubbing
- - Cancers: osteosclerotic myeloma, Castleman's, plasmacytoma
82
What is the chromophore for most of UVB's effects?
DNA
83
What is the UVB signature mutation? Most common DNA photoproducts (in order)?
Cytosine --> Thymine
TT > CT > TC and CC = cyclobutane-pyrimidine dimers
84
Cytokine expression induced by UVR?
Increased IL-1, IL6, IL-10, TNF-alpha
85
Role of UVA in...
- - sunburn
- - skin penetration
- - darkening
- - drug-induced photosensitivity
- - carcinogenesis
- - Vit D3 production
- - Glass penetration
- - Sunburn: minor role; causes immediate erythema and delayed erythema 6-24 hours later
- - Penetrates to deep dermis
- - Darkening: immediate pigment darkening due to melanin oxidation
- - Major contributor to drug-induced photosensitivity
- - Carcinogenesis: minor role; causes ROS production
- - NO role in vitamin D production
- - Penetrates window glass
* causes phytophotodermatitis
* causes photoaging
86
Role of UVB in...
- - sunburn
- - skin penetration
- - darkening
- - drug-induced photosensitivity
- - carcinogenesis
- - Vit D3 production
- - Glass penetration
- - Sunburn: Major role 6-24 h later; UVB 1000X more erythemogenic than UVA and causes apoptotic "sunburn" cells
- - Penetrates epidermis only
- - Darkening: delayed melanogenesis 48-72h later due to increasing melanocyte # and size of melanosomes, increase transfer of melanin
- - Minor role in drug-induced photosensitivity
- - Major role in carcinogenesis!
- - Major role in vitamin D metabolism
- - NO glass penetration
87
Common site for Juvenile Spring Eruption?
Helices
| *PMLE variant in boys
88
What are findings of photo testing in PMLE?
React to UVA > UVB
MEDB and MEDA are normal!
89
What differentiates Actinic Prurigo from Juvenile Spring Eruption?
-- Treatment
* Cheilitis and Conjunctivitis seen in Actinic Prurigo; typically Native Americans; lips can have lymphoid nodules
* Thalidomide is only effective systemic agent
90
Which drugs can cause photo recall reaction?
MTX, doxorubicin, daunorubicin, idarubicin, 5-FU, etoposide, taxanes, hydroxyurea
91
Phototesting findings for Chronic Actinic Dermatitis? Typical patient?
Most sensitive to UVB > UVA > visible light
| *Older men of darker skin types
92
UVA blockers (4)?
Avobenzones
Benzophenones (oxybenzone)
Ecamsule (Mexoryl SX)
Meradimate
93
UVB Blockers (7)?
```
PABA
Padimate O
Cinnamates
Salicylates
Octocrylene
Ensulizole
Mexoryl XL (drometrizole trisiloxane)
```
94
UVA and UVB Blockers (4)?
Titanium dioxide
Zinc oxide
Mexoryl XL
Oxybenzone
95
What does SPF mean?
MED with sunscreen / MED without sunscreen
Amt UVB absorbed by skin with SPF 50 on...
UVB = 100 - (1/SPF)
96
Fitzpatrick Skin Types
```
I = always burns, never tans
II = usually burns, rarely tans
III = rarely burns, usually tans
IV-VI = never burns, always tans
```
97
To be diagnosed with PCOS a patient must have 2 of the following 3 criteria...
1. Amenorrhea or oligomenorrhea ( 3 in most patients
3. Ultrasound findings consistent with PCOS
- - Antral follicle count > 12, ovarian volume > 10 ml
98
Endocrine labs to check if concerned for PCOS and expected results
* Testostorone (free and total): > 200 ovarian tumor; > 150 hyperandrogenism
* DHEA-S: subset of patients have normal testosterone but elevated DHEA-S (thinner patients typically)
* 17-OH-progesterone > 200 in PCOS
* Androstenedione
* LH:FSH > 3:1 is suggestive of PCOS
* Prolactin
* TSH - rule out thyroid disease as cause of menstrual irregularities
99
Clinical phenotype of someone with anti-155 (TIF1-gamma) antibodies.
Dermatomyositis with cutaneous ulcerations, oral pain/ulcerations, hand/foot swelling and rapidly progressive ILD
100
Clinical phenotype of someone with antisynthetase antibodies.
Name an anti-synthetase antibody?
anti-Jo
raynaud's, mechanic's hands, pulmonary fibrosis (ILD), arthritis, myositis
101
Clinical phenotype of someone with antiMi-2 antibodies?
Classic skin findings: gottron's papules, shawl sign & good prognosis
102
What antibodies is associated with cardiac involvement of dermatomyositits?
Anti-SRP
103
What are dermatoscopic features of melanoma (8)?
```
Blue-white veil
Pseudopods
Radial streaming
Scar-like depigmentation
Peripheral black dots/globules
Broad network
Negative network
Irregular vascularity
```
104
What is Darrier-Roussy Disease?
Sarcoidal panniculitis -- no epidermal involvement. Presents with painless subcutaneous mobile nodules.
Differentiated from Lofgren's syndrome, which is acute sarcoidosis w/ erythema nudism, hilar adenopathy, iritis, polyarthritis and fever
105
What is Heerfordt's syndrome?
Uveoparotid fever -- form of sarcoid with fever, parotid gland enlargement, anterior uveitis, facial nerve palsy
106
What wavelengths are UVA1, UVA2, UVB, UVC?
UVA1: 340-400 nm
UVA2: 320-340 nm
UVB: 290-320 nm
UVC: 200-290 nm
107
UVB induced sunburn reaches peak when? What is chromophore?
6-24 hours after exposure. Note that UVB does not cause immediate erythema. DNA is chromophore. UVB is major cause of sunburn!
108
Does UVA cause immediate / delayed erythema?
Both -- immediate erythema can occur; delayed erythema can occur 6-24 hours after exposure. Note that doses of UVA in natural sunlight are not sufficient to induce a sunburn.
109
Immediate pigment darkening is caused by? Delayed tanning is caused by?
Immediate pigment darkening: UVA-induced, oxidation and redistribution of existing melanin.
Delayed pigment darkening: UVB-induced, peaks 3 days after sun exposure -- increased number of melanocytes, increased melanin synthesis, increased arborization of melanocytes, increased melanosome transfer to keratinocytes
110
How does UVB make vitamin D?
UVB converts epidermal 7-dehydrocholesterol --> previtamin D3 --> isomerization to vitamin D3 (cholecalciferol) --> converted to 25-hydroxyvitamin D in liver --> 1,25 dihydroxyvitamin D in kidneys
111
What are the 3 key features of actinic prurigo? HLA association? tested treatment?
3Cs: crusting, cheilitis, conjunctivitis
HLA-DR4
Treatment: thalidomide
112
Patients with chronic actinic dermatitis may have positive patch test results to...(3)
Compositae, musk ambrette, sunscreen
| Also have positive photo patch test results (atopic dermatitis would not)
113
What is the action spectrum of solar urticaria?
Most often visible light but can also be UVA, UVB or a combination. This is why sunscreen is often not helpful -- it does not block UVA or visible light.
114
What is the most common cause of photo allergic reaction in the United States?
Sunscreen, specifically Benzophenone
115
What conditions are associated with erythema nodosum (10)?
"SHOUT BCG"
- Strep / Sarcoid / Sulfa
- Histoplasmosis
- OCP
- Ulcerative colitis
- TB
- Behcet's
- Crohns (more often than UC)
- Gastroenteritis
116
Which medications are associated with pseudoporphyria (many)?
NSAIDS (naproxen classically but nearly all reported), diuretics (furosemide, HCTZ, torsemide, butamide, others), retinoids (isotretinoin, etretinate), UV light (sun, phototherapy, tanning bed), antibiotics (tetracyclines, cipro, others), random meds (dapsone, cyclosporine, B6, IV 5-FU, OCPs, coca-cola, voriconazole, imatinib)
