Group 1, Column 1 Flashcards

1
Q

Name the diagnosis.

  • What is the most common associated malignancy?
  • This is histologically identical to what three other things?
  • How do you treat this?
A

Acanthosis nigricans

  • Most common underlying malignancy is GI adenocarcinoma, especially with palmar involvement
  • Derm path buzzword: epidermal papillomatosis
  • Histologically identical to CARP, acrokeratosis verruciformis of Hopf and SK
  • Can be treated with topical retinoids and AmLactin
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2
Q

What are the four components of acne vulgaris?

A
  1. Abnormal follicular keratinization
  2. Sebum overproduction
  3. Propionibacterium acnes overgrowth
  4. Inflammation
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3
Q

Topical retinoids downregulate which receptor?

A

TLR-2

P. acnes activates this on macrophages, causing inflammation and attracting neutrophils

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4
Q

Describe key findings of actinic keratosis (AK).

  • What kind of genetic damage/mutation has occurred?
A
  • UVB causes AKs by inducing thymidine dimers and causing p53 mutations, impairing apoptosis
  • Look for “flag sign” with overlying parakeratosis (looks pink) alternating with orthohyperkeratosis (looks blue)
  • Rate of transformation to SCC: less than 0.1%
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5
Q

What kinds of immune cells target the hair follicles in alopecia areata and where?

What type of cytokines are involved?

A

CD8+ T-cells attack bulb, involving type 1 cytokines (IL-2, IL-12, IFN-gamma and TNF-alpha)

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6
Q

What is important to know about the prognosis of ophiasis pattern alopecia areata?

A

There is a poor prognosis.

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7
Q

What is the opposite of ophiasis pattern?

A

Sisapho pattern

(This is basically ophiasis spelled backward.)

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8
Q

What are the nail findings in alopecia areata?

A
  • Regular (geometric) nail pitting
  • Trachyonychia (or 20-nail dystrophy), where nails are sandpaper-like
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9
Q

What findings on dermoscopy are classically seen with alopecia areata?

A
  • Exclamation point hairs
  • Perifollicular yellow dots
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10
Q

What are other disease associations with alopecia areata?

A
  • Atopy (a poor prognostic factor)
  • Autoimmune thyroid
  • Vitiligo
  • SLE
  • IBD
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11
Q

What are classic histology findings in alopecia areata?

  • What kind of inflammatory pattern is seen?
  • What kind of hairs are present?
A
  • Peribulbar lymphocytic infiltrate (“swarm of bees”)
  • Catagen-telogen shift
  • Increased miniaturized hairs (i.e., nanogen hairs)
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12
Q

What does anagen mean?

  • What proportion of hairs are in this phase?
  • How long does this last?
  • What medicine recreates this phase?
A
  • Hair growth phase and determines length of hair
  • 85% of hairs at any particular time
  • Lasts 2-6 years (genetically determined)
  • Minoxidil recreates anagen phase
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13
Q

What does catagen mean?

  • What happens to the hair in this phase?
  • What proportion of hair are in this phase?
  • How long does this last?
A
  • Transitional phase after anagen
  • The bulb regresses and the inner root sheath (IRS) is lost
  • 2% of hairs at any particular time
  • Lasts 2-4 weeks
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14
Q

What does telogen mean?

  • How long does this last?
  • What proportion of hairs are in this phase?
A

“Telogen is tired and resting.”

  • Resting phase
  • Lasts 3 months
  • 15% of hairs at any particular time
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15
Q

Name the layers of the hair follicle from outer to inner.

A
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16
Q

What is the gene implicated in atopic dermatitis?

A

Fillagrin mutations, causing alterations in the epidermal barrier

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17
Q

Where is filaggrin located in the epidermal cell and in what layer of the epidermis?

  • What does filaggrin do in the cell?
A
  • In the keratohyalin granules of the stratum granulosum
  • Filaggrin binds to intermediate filaments, also known as keratin filaments, causing structural integrity
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18
Q

Is atopic dermatitis Th1 or Th2 mediated?

A

Th2

  • Important in allergies, humoral immunity
  • Think IL-4, IL-5, IL-6, IL-10, IL-13
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19
Q

Name the Th1 cytokines.

A
  • IL-2
  • IL-12
  • IFN-γ (downregulates Th2 pathway)
  • TNF-α

Important in cell-mediated immunity, delayed-type hypersensitivity reactions

Think ACD, psoriasis

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20
Q

Name the Th2 cytokines.

A
  • IL-4
  • IL-5
  • IL-6
  • IL-10
  • IL-13

Important in allergies, humoral immunity

Think atopic dermatitis

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21
Q

Name the diseases associated with Th1 cytokine profile.

A
  1. Allergic contact dermatitis
  2. Psoriasis
  3. Tuberculoid leprosy
  4. Cutaneous leishmaniasis
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22
Q

Name the diseases associated with Th2 cytokine profile.

A
  • Atopic dermatitis
  • Lepromatous leprosy
  • Disseminated leishmaniasis
  • Sezary syndrome
  • Parasitic infections
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23
Q

What is the classic histopathology of acute atopic dermatitis?

  • What happens in the epidermis?
  • What kind of inflammatory pattern and what immune cells are present?
A
  • Prominent spongiosis
  • Intraepidermal vesicles/bullae
  • Perivascular lymphohistiocytic infiltrate with eosinophils
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24
Q

What is the classic histopathology of chronic atopic dermatitis?

  • What happens in the epidermis?
A
  • Psoriasiform acanthosis with little spongiosis
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25
What are the two most common genetic mutations in **BCCs**?
* ***PTCH* (chromosome 9q)**, most common * p53 point mutations, second most common
26
List the (8) subtypes of **BCCs**
1. Nodular 2. Superficial 3. Morpheaform/sclerotic 4. Micronodular 5. Fibroepithelioma of Pinkus 6. Pigmented 7. Infundibulocystic 8. Basosquamous
27
List the genetic syndromes associated with multiple **BCCs**. Hint: "**G**reen **B**erets **R**arely **B**uy e**X**tra **S**hoes"
"**G**reen **B**erets **R**arely **B**uy e**X**tra **S**hoes...but they get a lot of BCCs from being in the sun!" * **G**orlin's * **B**azex-Dupre-Christol * **R**ombo * **B**rooke-Spiegler * **X**eroderma pigmentosum * **S**chopf-Schulz-Passarge
28
What is the classic histology of **BCC**? * What do the cells look like? How do they organize? How do they interact with the stroma?
* **Nests of basaloid and uniform cells** with high N:C ratio (i.e., **look blue**) * **Peripheral palisading** * At least focal epidermal connection * **"Retraction spaces"** between epithelium and stroma
29
What is the chemotherapy used for inoperable or metastatic **BCC**?
**Vismodegib**, a hedgehog pathway inhibitor (and the first of its kind)
30
Describe **_candidal_ diaper dermatitis**. * Are the intertriginous areas and scrotum involved? * What does Candida look like on histology?
* Bright red patches with pustules and satellite papules with **possible intertriginous involvement, including the scrotum** * May also have thrush * Look for pseudohyphae and budding yeast on histology
31
What are the two most common causes of **cellulitis**?
**Group A beta-hemolytic strep (GAS)** Followed by *S. aureus* (the most common cause in children)
32
What are the (3) antibiotics of choice for uncomplicated cases of **cellulitis**?
* Oral **cephalexin**, **dicloxacillin** or **clindamycin** for 10 days
33
What is the antibiotic of choice for **cellulitis** associated with decubitus or diabetic ulcers? (2 regimens) * What kinds of bacteria must these antibiotics cover?
**Piperacillin-tazobactam (Zosyn) or ciprofloxacin-metronidazole (Flagyl)** This covers GPs, GNs and anaerobes, as these ulcers tend to be polymicrobial.
34
What is the antibiotic of choice for **MRSA cellulitis**? (4)
* Bactrim * Minocycline * Doxycycline * Clindamycin
35
State the one-liner for hypersensitivity reactions: * Type I * Type II * Type III * Type IV
* Type I: IgE-mediated (anaphylaxis) * Type II: IgG-mediated cytotoxic (i.e., autoimmune hemolytic anemia, Goodpasture syndrome, myasthenia gravis) * Type III: immune complex-mediated * Type IV: delayed, cell-mediated
36
What is the most common cause of **allergic contact dermatitis** in the U.S. and the world?
* US: poison ivy * Worldwide: nickel
37
What is the classic histology of **ACD**? * What is the inflammatory pattern, and what cells are notably present in the dermis? * Are necrotic keratinocytes present?
* Spongiotic dermatitis with **dermal eosinophils** * **Lacks necrotic keratinocytes** (found in ICD)
38
What is the classic histology of **ICD**? * What is the inflammatory pattern, and what cells are notably present in the dermis? * Are necrotic keratinocytes present?
* Mild spongiosis * **Scattered necrotic keratinocytes (in contrast with ACD)** * Mild perivascular inflammation * Probably not eosinophils (compared to ACD)
39
How do you discriminate between an **irritant and allergic** response on patch testing?
* Irritant reactions will fade between the first and second readings * Allergic reactions will persist or progress
40
Name the diagnosis.
**Dermatofibroma**
41
Name the diagnosis.
**Dermatomyositis** * Gottron's papules are over MCP joints * Versus Gottron's sign (which is reddish scaling over knuckles, knees and elbows)
42
Name the diagnosis.
**Dermatomyositis (shawl sign)**
43
Name the diagnosis. * What are three nail findings of these condition?
**Dermatomyositis** * Ragged cuticles (Samitz sign) * Nailfold telangiectasias * Periungual erythema
44
Name the diagnosis.
**Dermatomyositis (heliotrope rash)**
45
Name the diagnosis.
**Dermatomyositis** | (Ragged cuticles, a.k.a. "Samitz sign")
46
Name the diagnosis.
**Dermatomyositis** * Gottron's sign (reddish scaling over knuckles, knees and elbows)
47
Name notable causes of **_drug-induced_ dermatomyositis**.
* **Hydroxyurea** (a chemotherapy for SCC and treatment for sickle cell disease; most common, \> 50% of cases) * **Statins** * Cyclophosphamide * TNF-alpha inhibitors
48
Describe the classic muscle and activity problems in **dermatomyositis**.
* Symmetric proximal muscle weakness **without myalgias** * Difficulty walking up stairs or brushing hair
49
Describe the classic skin findings of **dermatomyositis**. Hint: think about face, chest and arms.
* Gottron's papules (lichenoid papules overlying knuckles) * Gottron's sign (maculary erythema overlying joints) * Facial erythema with malar involvement _AND melolabial involvement_ (versus lupus) * Heliotrope sign (lid erythema and edema) * Holster sign (erythema of lateral hips/thighs, see photo) * V-sign/"shawl" sign * Poikiloderma
50
Why does the **heliotrope sign** arise?
It is the result of inflammation of **underlying orbicularis oculi muscle** and NOT the skin.
51
What is **mechanic's hands of dermatomyositis** associated with?
Anti-synthetase syndrome
52
What are the notable nail changes of **dermatomyositis**?
* Ragged cuticles (Samitz sign) * Proximal nail fold with dilated capillary loops, as well as other areas with vessel dropout * Periungual erythema
53
What is one way to help differentiate **dermatomyositis** from lupus or psoriasis based on _pruritus_?
**Dermatomyositis is often very pruritic** (especially on the scalp), while the others are usually NOT
54
What are the associated malignancies of **dermatomyositis** in men and women?
* Lung and GI cancer most common in men * Ovarian and breast most common in women
55
Name the findings of **anti-synthetase syndrome**. Hint: think about clinical findings, symptoms and rheumatologic serologies.
* **Mechanics hands** * **ILD** (pulmonary fibrosis) * Antisynthetase autoantibodies (_Anti-Jo1_) * Acute disease onset (myositis) * Constitutional symptoms * Raynauds * Arthritis
56
Name the diagnosis. * What is the buzzword for this diagnosis? * In patients with which other skin conditions is this diagnosis seen?
**Eczema herpeticum** * Monomorphic, umbilicated vesiculopapules or _punched-out ulcerations_ * Disseminated HSV (usually HSV-1) mainly seen in atopics, Darier, Hailey-Hailey
57
What is the classic histopathology of **dermatomyositis**? * What is the inflammatory pattern? What is abundant in the dermis?
* Subtle vacuolar interface dermatitis * Very abundant mucin * Mild inflammation
58
What are the most common causes of **erythema multiforme**?
**HSV is by far the most common cause of EM (HSV-1 \> HSV-2) in general** * HSV is the most common cause of EM minor * Mycoplasma pneumoniae is the most common cause of EM major (simulates SJS)
59
Name the diagnosis.
**Erythema multiforme**
60
Describe the three zones of a typical target lesion of **EM**.
**Three zones** 1. A dusky, vesicular or necrotic center 2. Elevated, edematous pale surrounding ring 3. Outer rim of macular erythema
61
Describe the papular (elevated) atypical target lesion of **EM**.
**Only two zones, but is palpable** * Note that _macular_ atypical lesions are seen in SJS/TEN but NOT EM
62
Describe the components of **EM minor**.
* Targetoid lesions (palpable) * Minimal mucosal involvement * No systemic symptoms
63
Describe the components of **EM major**.
* Target lesions (palpable) * Severe mucosal involvement (mouth \> eyes \> genitals) * **Systemic symptoms** (fever, arthralgias)
64
What infectious lab testing is often positive in **EM** patients?
**80%** have detectable HSV DNA by PCR in early erythematous papules or outer rim of targets
65
What is the classic histopathology of **EM**? * What is the inflammatory pattern? * Where is the inflammatory infiltrate? * Are necrotic keratinocytes and eosinophils present?
* Prominent basal vacuolar change and spongiosis * Moderately dense superficial dermal perivascular lymphohistiocytic infiltrate * Necrotic keratinocytes * Absent/rare eosinophils (as opposed to SJS/TEN)
66
Name the diagnosis.
**Granuloma annulare**
67
What is the classic histopathology for **granuloma annulare**? * What is more abundant in the dermis? * Are eosinophils present?
* Granulomas in the dermis * Increased mucin * Scattered eosinophils
68
Describe the three common histologic patterns of **GA**.
1. **Interstitial** (most common; 70% of cases): subtle, singly-arrayed histiocytes between collagen fibers intermixed with increased dermal mucin, perivascular eosinophils 2. **Palisaded granulomas** (25%) 3. **Sarcoidal pattern** (5%)
69
What is the classic histopathology of **HSV**? * What are the epidermal changes? What is the buzzword for the keratinocyte change? * What is the name of the viral inclusions seen within keratinocyte nuclei?
* **Intraepidermal vesicles** with **slate-gray enlarged keratinocytes (ballooning degeneration)** that are multinucleated * +/- Cowdry A inclusions (eosinophilic) within nucleus
70
Name the diagnosis.
**HSV** "Dew drops on a rose petal"
71
What do you use to treat **eczema herpeticum, neonatal HSV, or severe HSV in immunosuppressed**? * What do you use in refractory cases?
* **First line: IV acyclovir** * If acyclovir-resistant, use foscarnet or cidofovir
72
What constitutes **disseminated herpes zoster**? * What patients tend to have this? * What other organs may be affected when this occurs?
* **Dermatomal disease and \> 20 lesions outside** of the dermatome * Seen in the immunosuppressed * Increased risk of pneumonitis and encephalitis
73
Describe **Ramsey-Hunt syndrome**. * What are the cause and clinical symptoms?
* Reactivation of varicella infection * Geniculate ganglion of **CN VII** is affected * Ipsilateral facial nerve paralysis * Hearing loss * Anterior 2/3 tongue taste loss
74
What is **Hutchinson's sign**? * What is involved/affected? * What does this raise special concern for?
* **Involvement of the side and tip of nose** with herpes zoster, indicating disease of **CN V1 (ophthalmic nerve)** * Raises concern for **uveitis and vision loss**
75
Name the diagnosis.
**Bullous impetigo**
76
Name the diagnosis.
**Impetigo**
77
What is the pathophysiology of **bullous impetigo**? * What are the toxins involved and desmoglein is targeted?
Phage group II (types 55 and 77) S. aureus --\> produce **exfoliatoxins A and B** --\> cleaves **desmoglein 1** --\> subcorneal/intragranular acantholysis
78
True or false? **Streptococcal impetigo** can lead to **rheumatic fever**.
**False** **_Only_ streptococcal pharyngitis** leads to rheumatic fever.
79
**Bullous _impetigo_** plus renal insufficiency or immunodeficiency can lead to what serious condition? Why?
**Staph scalded skin syndrome** * Due to disseminated exfoliatoxin
80
**_Bullous impetigo_** has nearly identical histology with which other disorder because the target is the same?
**P. foliaceus** Both involve cleavage of **desmoglein 1**
81
Name the diagnosis.
**Keloid**
82
What is the histopathology of **lentigo simplex**? * What are the epidermal changes?
* Basal layer hyperpigmentation * Elongated rete ridges with mild increased melanocyte density
83
What (6) conditions are associated with **multiple lentigines**?
1. LEOPARD 2. Carney complex (LAMB/NAME) 3. Peutz-Jeghers (especially oral/perioral) 4. Bannayan-Riley-Ruvalcaba (penile) 5. Cowden syndrome 6. Xeroderma pigmentosum
84
Name the diagnosis.
**Lentigo simplex**
85
Name the diagnosis.
**Lichen planus**
86
What are classic _viral_ triggers of **lichen planus**?
* **HBV** (oral or bullous LP) * **HCV** (oral ulcerative/erosive LP)
87
Name the diagnosis.
**Lichen planus**
88
What are classic _contact allergen_ triggers of **LP**? (3)
* Mercury amalgam (tooth fillings; oral LP) * Gold * Copper
89
Name the diagnosis.
**Lichen planus**
90
What are classic triggers of **drug-induced LP**?
* **HCTZ (often photo-exacerbated)** * Beta-blockers * ACE inhibitors * Antimalarials * "Gold salts" (used for RA) * Note that **ACE inhibitors, beta-blockers and gold salts** are also drug triggers of **pityriasis rosea**.
91
What is the classic histopathology of **LP**? * How are the granular layer and rete ridges affected? * What are the special cells found in the superficial dermis? * Are eosinophils and parakeratosis present?
* **Wedge-shaped hypergranulosis** * **Saw-toothed rete ridges** * Apoptotic keratinocytes of basal layer with some falling into superficial dermis (eosinophilic, a.k.a. ***civatte or colloid bodies***) * **Usually lacks eosinophils and parakeratosis** (except drug-induced LP) * "Shaggy" fibrinogen along BMZ
92
Name the diagnosis and nail finding.
**Lichen planus (dorsal pterygium)** * Can also see 20-nail dystrophy (trachyonychia; children \>\> adults) in LP
93
Name the diagnosis.
**Livedo reticularis**
94
What inherited genetic mutation raises risk for **familial melanoma**? * What syndromes is this seen in? (2) * What tumor suppressor genes are involved? (2)
Inherited **CDKN2A mutations** Seen in **FAMMM syndrome**, **dysplastic nevus syndrome** * Tumor suppressor genes **p14 (via p53)** and **p16 (via Rb)** stop working
95
Name the diagnosis.
**Melanoma**
96
Name the four subtypes of **melanoma**.
1. Superficial spreading 2. Nodular 3. Lentigo maligna 4. Acral lentiginous
97
What is the most common subtype of **melanoma**?
**Superficial spreading**
98
What are factors associated with a poor prognosis for **melanoma**? * Some are clinical and some are histologic
Clinical: * Ulceration * Lymph node or visceral metastasis * Head/neck/trunk location Histologic: * Breslow \> 1 mm * Increased mitotic rate
99
Name this clinical sign. * What condition is this seen in?
* **Hutchinson's sign**: extension of pigment from the nail bed, matrix, and nail plate to the adjacent cuticle and/or nail folds * Seen in nail matrix lesions of **acral lentiginous melanoma**
100
What are indications for **sentinel lymph node biopsy**?
* **Breslow \> 1 mm** (which is why you want a biopsy at least 1 mm deep) * **Ulcerated** * **\>/= 1 mitosis**
101
Name the diagnosis.
**Discoid lupus** (Chronic cutaneous lupus erythematosus, CCLE) * Red macules and plaques develop scale, **atrophy and scarring** * **Central hypopigmentation** * **Peripheral hyperpigmentation**
102
Name the diagnosis.
**Discoid lupus** (Chronic cutaneous lupus erythematosus, CCLE) * Red macules and plaques develop scale, **atrophy and scarring** * **Central hypopigmentation** * **Peripheral hyperpigmentation**
103
Name the diagnosis.
**Discoid lupus** (Chronic cutaneous lupus erythematosus, CCLE) * Red macules and plaques develop scale, **atrophy and scarring** * **Central hypopigmentation** * **Peripheral hyperpigmentation**
104
Name the diagnosis. * What is there an increased risk for? * What part of the body is favored in this condition, compared with another similar condition?
**Hypertrophic (verrucous) lupus erythematosus** * **Thick, hyperkeratotic and verrucous scaling plaques** with indurated border * **Increased risk of SCC** * **Favors upper half of body** (versus hypertrophic LP, which favors lower half) * Usually accompanies typical discoid lupus lesions
105
Name the diagnosis. * What can trigger this?
**Chilblains lupus erythematosus** * Red or dusky purple papules and plaques on **fingertips, rims of ears**, heels and **toes** * **Precipitated by cold, moist climates but can persist year-round** * Note: Chilblains can occur by itself (without lupus)
106
Name the diagnosis. * This looks similar to what other condition?
**Tumid lupus erythematosus** * Edematous, indurated, erythematous, often **annular or arcuate plaques without epidermal change** * Favors face and trunk * Similar in appearance to **Jessner's lymphocytic infiltrate** (see photo)
107
What is the classic histopathology of **discoid lupus**? * What happens in the epidermis? * What is the inflammatory pattern? * Where is the infiltrate and what composes it? * Is follicular plugging present? * What is present in the stroma?
* **Epidermal atrophy** * **BMZ thickening** * **Vacuolar interface dermatitis** * **Follicular plugging** * Superficial and deep **perivascular and periadnexal lymphohistiocytic inflammation** with plasma cells * **Mucin** deposition * **DIF: Lupus band test positive** (IgG and complement deposits at DEJ)
108
What is the suspected pathogenesis of **subacute cutaneous lupus erythematosus (SCLE)**?
* **UVR-induced apoptosis releases high levels of nuclear antigens** (Ro, La, DNA) * **Reduced clearance of apoptotic cells** leads to loss of immune tolerance * Release of pro-inflammatory cytokines and production of ANAs (especially **anti-Ro/La auto-Ab**)
109
What are genetic associations with **SCLE**?
* **HLA-B8 (strongest association)**, HLA-DR3 * **Hereditary complement deficiencies** (especially those involving the early intrinsic pathway: C1q/r/s, C2 and C4)
110
What _two_ autoimmune diseases are strongly associated with **anti-Ro/SS-A autoantibodies**?
1. Sjogren syndrome 2. Subacute cutaneous lupus erythematosus (SCLE)
111
Name the diagnosis. * What are the two clinical variants of this condition? * What parts of the body are usually involved?
**SCLE** * May occur as two clinical variants: * **Papulosquamous SCLE** (psoriasiform plaques) * **Annular SCLE** (scaly polycyclic annular plaques with central clearing) * Involves sun-exposed areas of **lateral face (central face spared), V-chest, upper back,** neck and extremities * **Arthritis/arthralgias** is most common systemic finding (up to 70%)
112
Name the diagnosis. * What are the two clinical variants of this condition? * What parts of the body are usually involved?
**SCLE** * May occur as two clinical variants: * **Papulosquamous SCLE** (psoriasiform plaques) * **Annular SCLE** (scaly polycyclic annular plaques with central clearing) * Involves sun-exposed areas of **lateral face (central face spared), V-chest, upper back,** neck and extremities * **Arthritis/arthralgias** is most common systemic finding (up to 70%)
113
Name the diagnosis. * What are the two clinical variants of this condition? * What parts of the body are usually involved?
**SCLE** * May occur as two clinical variants: * **Papulosquamous SCLE** (psoriasiform plaques) * **Annular SCLE** (scaly polycyclic annular plaques with central clearing) * Involves sun-exposed areas of **lateral face (central face spared), V-chest, upper back,** neck and extremities * **Arthritis/arthralgias** is most common systemic finding (up to 70%)
114
What rheumatologic serologies are found in **SCLE**? * Also, what else might be abnormal on CBC?
* **Anti-Ro/SS-A (75% - 90%)** * **Anti-La/SS-B (30% - 40%)** * ANA (60% - 80%): speckled/particulate pattern * Leukopenia (20%)
115
What is the classic histopathology of **SCLE**? Hint: Think about its findings regarding the corneum, epidermis, dermatitis pattern, and whether mucin, eosinophils and follicular plugging are present.
* Compact hyperkeratosis * **Prominent epidermal atrophy** * **Vacuolar interface dermatitis** with pigment incontinence * Perivascular and periadnexal lymphoid aggregates of the superficial dermis with scattered plasma cells * **Mucin deposition** * Lacks eosinophils and follicular plugging * DIF: Positive lupus band test in 60 - 80%
116
What are the first and second line treatments for **SCLE**?
* First line: **TCS, antimalarials (hydroxychloroquine-quinacrine; requires annual eye exams)** and sun protection * Second and third line: **oral retinoids (acitretin, isotretinoin)**, dapsone, methotrexate, mycophenolate mofetil
117
What are the most important implicated drugs of **drug-induced SCLE**?
* **HCTZ (most common; same as drug-induced LP)** * **Terbinafine** * **Griseofulvin** * **Calcium channel blockers** * ACE inhibitors * TNF-alpha inhibitors (most commonly etanercept) Think HCTZ, oral antifungals and CCBs.
118
Name the diagnosis. * Are the lesions usually present at birth? * What is this condition the result of? * The baby should be evaluated for what other serious congenital condition?
**Neonatal lupus erythematosus** * Lesions arise within first weeks of life but not at birth * Photosensitivity * Periorbital erythema = **"raccoon eyes"** * **Annular, polycylic erythematous plaques with central clearing** and raised red border, fine scale favoring head and neck (looks similar to SCLE) * **Resolves without scarring but with dyspigmentation and telangiectasias by around 6 months old** * Result of transplacental passage of anti-Ro/SS-A autoantibodies, which can lead to **congenital third-degree heart block**
119
Name the diagnosis. * What other parts of the body are classically involved with this rash?
**Acute cutaneous lupus (ACLE)** * **Most strongly associated with systemic lupus erythematosus (SLE)** of all cutaneous subtypes * Malar erythema ("butterfly rash") for hours to weeks after sun exposure * Classically involves the nasal bridge and bilateral malar eminence * **Sparing melolabial folds (in contrast to facial erythema of dermatomyositis)** * May develop scaling, papules, erosions, **poikiloderma** * May be accompanied by generalized photodistributed eruption of the **V-neck, upper back, extremities but _classically sparing the knuckles_ (unlike dermatomyositis)**
120
Name the diagnosis. * What other parts of the body are classically involved with this rash?
**Acute cutaneous lupus (ACLE)** * **Most strongly associated with systemic lupus erythematosus (SLE)** of all cutaneous subtypes * Malar erythema ("butterfly rash") for hours to weeks after sun exposure * Classically involves the nasal bridge and bilateral malar eminence * **Sparing melolabial folds (in contrast to facial erythema of dermatomyositis)** * May develop scaling, papules, erosions, **poikiloderma** * May be accompanied by generalized photodistributed eruption of the **V-neck, upper back, extremities but _classically sparing the knuckles_ (unlike dermatomyositis)**
121
What is the classic histopathology of **acute cutaneous lupus erythematosus** (ACLE)? Hint: think about what the dermatitis pattern is and where/what the infiltrate is.
* **Vacuolar interface dermatitis** * **_Sparse_ perivascular lymphocytic infiltrate limited to upper dermis** * Dermal edema * DIF: Positive lupus band test
122
Name the diagnosis. * What autoantibody is involved? * What parts of the body may be involved with this rash? * What is the treatment?
**Bullous SLE** * **Anti-type VII collagen autoantibodies (same as EBA)** * **Widespread, symmetric eruption of dense, subepidermal bullae on erythematous-to-urticarial base** * Involves both **sun-exposed and non-exposed areas, including the mucosa** * Patients must meet ACR criteria for SLE to term it bullous SLE * **Treat with dapsone**
123
Name the diagnosis. * What autoantibody is involved? * What parts of the body may be involved with this rash? * What is the treatment?
**Bullous SLE** * **Anti-type VII collagen autoantibodies (same as EBA)** * **Widespread, symmetric eruption of dense, subepidermal bullae on erythematous-to-urticarial base** * Involves both **sun-exposed and non-exposed areas, including the mucosa** * Patients must meet ACR criteria for SLE to term it bullous SLE
124
What is the classic histopathology of **bullous SLE**? * What occurs at the DEJ? * What is found on DIF?
* **Subepidermal bullae with neutrophils at DEJ and dermal papillae** * DIF: granular to linear deposition of IgG, IgM, IgA and/or C3 along BMZ (also called "a full house")
125
What laboratory testing is notable in **bullous SLE**? * What is found on salt-split skin testing? * An ELISA tests for what autoantibodies? * Is ANA positive?
* **Dermal reactivity (i.e., floor pattern)** on salt-split skin * ELISA: autoantibodies to **type VII collagen (same as EBA)** * **ANA positive**
126
What is the treatment of choice for **bullous SLE**? * A quick response differentiates it from what other condition?
**Dapsone** * **Dramatic response within 1-2 days**, differentiating bullous SLE from EBA
127
What are the general ACR criteria for diagnosis of **systemic lupus erythematosus (SLE)**? Hint: think about the clinical, immunologic and serologic features, as well as a biopsy of a specific organ.
Need to satisfy **four items** (at least one clinical and one immunological item) OR have biopsy-proven nephritis compatible with SLE in the presence of ANA or anti-dsDNA antibodies * Malar or discoid rash * Photosensitivity * Oral ulcers * Arthritis * Serositis (pleuritis, pericarditis) * Renal disorder (proteinuria, cellular casts) * Neurologic disorders (seizures, psychosis) * -cytopenias * Anti-DNA, Anti-Sm, antiphospholipid antibodies (i.e., anti-cardiolipin, lupus anticoagulant, false positive RPR) * ANA
128
How should pregnant women with **systemic lupus erythematosus** be managed in terms of medicine and clot prophylaxis?
* Continue with **hydroxychloroquine** and **low-dose steroids** * Consider azathioprine * Anticoagulation for antiphospholipid syndrome (APLS)
129
What laboratory testing is seen in **systemic lupus erythematosus**? Hint: think about how it affects a CBC with diff, complement levels, inflammatory markers, UA, as well as autoantibodies (5)
* Hemolytic anemia (Coombs positive) * -Cytopenias * Decreased total complement levels (CH50) * Increased inflammatory markers (CRP, ESR) * Proteinuria, hematuria * Anti-C1q Ab (associated with severe SLE nephritis) * ANA (99%) * Anti-dsDNA (60%; highly specific) * Anti-Smith (10% - 30%; highly specific) * Anti-histone (seen in drug-induced SLE)
130
What is the treatment for **mild SLE** _without_ visceral involvement?
**Hydroxychloroquine + NSAIDs**
131
What is the treatment for **moderate-to-severe SLE** _without_ renal involvement?
**Prednisone + steroid-sparing agent (e.g., azathioprine, MTX or MMF)**
132
What is the treatment for **severe SLE** _with_ renal involvement?
**High dose prednisone + pulsed IV cyclophosphamide or MMF**
133
What is the treatment for **moderate-to-severe and recalcitrant SLE**?
**Rituximab** or **belimumab**
134
How long after drug initiation does **drug-induced lupus** classically occur? * How long does it take to resolve?
* Onset is **usually \> 1 year** after drug initiation * Typically resolves **within 4 - 6 weeks of stopping the drug**
135
What rheumatologic serologies characterize **drug-induced SLE**? (2)
* **Positive anti-histone** antibody (\> 95%) * **Negative dsDNA** antibody
136
What are the most important implicated drugs for **drug-induced lupus**? Hint: for high risk medications, think about the heart.
* **High risk: procainamide, hydralazine** * Medium to low risk: methyldopa, isoniazid, D-penicillamine, minocycline, TNF-alpha inhibitors
137
In children, what are the most common causes of **urticaria**?
* Viral * Idiopathic * Infectious (UTI, URI, GI infection) * Allergic (foods, meds, environmental allergens) * Physical stimuli (pressure, solar, cold) * Arthropod bite reactions ("papular urticaria") * Malignancy (most commonly lymphoma)
138
What are the most common causes of **acute urticaria**?
* **Idiopathic (#1)** * **URIs (#2)** * Drugs (**beta-lactams** most common)
139
What are different types of **physical urticaria**?
* Dermatographism * Delayed pressure * Heat-induced * Cold ("ice cube test" for primary cold contact urticaria, PCCU) * Cholinergic (exercise, hot bath, emotions) * Adrenergic (reproduced by norepinephrine injection) * Solar * Aquagenic (associated with cystic fibrosis)
140
How can **urticaria** and **urticarial vasculitis** be distinguished? Hint: think about lesion duration, specific associated symptoms and histology.
* Any given urticarial lesion **lasts \< 24 hours** * **Urticarial vasculitis (UV) lesions last \> 24 hours** * **UV burns/hurts and leaves behind bruises** rather than itches (like urticaria) * **UV shows mild LCV** +/- eosinophils
141
Name the diagnosis (these lesions have been present for _\> 24 hours_). * What are associated symptoms, lab findings and treatments for this? * What broad family of diseases is associated with this diagnosis?
**Urticarial vasculitis** * **Pain/burning** \> itch (unlike acute urticaria) * Usually **chronic** * **Angioedema in one-third of patients** * **Arthralgias (50%)**, GI issues (20%), asthma (20%) * Increased ESR, decreased complement, (+) ANA * **Treat with NSAIDs (first line)**, colchicine, dapsone, MTX, steroids * Associated with **autoimmune CTDs**
142
What is the classic histopathology of **urticaria**?
* **Superficial dermal edema** * Vasodilation * Scant perivascular and **interstitial infiltrate** seen in lesions at least an hour old * **Predominantly neutrophils** ( \> eosinophils, lymphocytes)
143
What kind of testing can be performed for **acute and recalcitrant chronic urticaria**? Hint: think about specific allergy testing and blood work, including antibody testing.
* For acute urticaria: * Radioallergosorbent (**RAST**) test (20% false negative rate) * **Skin prick** (intradermal) test * For recalcitrant chronic urticaria * CBC with diff * ESR/CRP * **Anti-thyroid Ab**, TFTs * Anti-FcεRI and anti-IgE Ab
144
What is the treatment of **urticaria**? * What oral and topical medications can be used? * What can be used in recalcitrant cases? * What should be avoided?
* **First-line treatment = H1 antihistamines (cetirizine [Zyrtec], diphenhydramine [Benadryl])** * Recalcitrant cases: doxepin, PO steroids, montelukast, phototherapy, colchicine, dapsone, omalizumab * Soothing lotions (pramoxime, menthol) * Avoidance of triggers * Avoid aspirin, NSAIDs and opiates