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Bullous and Vesicular Dermatoses > Autoimmune Blistering Diseases > Flashcards

Autoimmune Blistering Diseases Flashcards

(43 cards)

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1
Q

Pemphigus vulgaris

-> Which signs positive?

A
  • > Nikolsky’s sign

- > Asboe-Hansen sign (blisters spread with pressure on surface of blister)

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2
Q

Pemphigus vulgaris

-> Histology?

A
  • > Suprabasilar acantholysis with “tombstoning” of basal layer
  • > Acantholysis can involve hair follicles and sebaceous glands
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3
Q

Pemphigus vulgaris

-> DIF pattern?

A

-> Intercellular IgG4 > C3, more prominent in lower epidermis

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4
Q

Pemphigus vulgaris

-> IIF substrate and pattern?

A
  • > Monkey esophagus
  • > Intercellular
  • > Can follow titers (dsg1, dsg3) for disease activity
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5
Q

Pemphigus vulgaris

-> Abs?

A
  • > Desmoglein 3 (mucosal)

- > Desmoglein 1 and 3 (mucocutaneous)

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6
Q

Pemphigus vulgaris

-> Drug induced?

A
  • > Pyrazolone derivatives
  • > Thiol-drugs
  • > Rifampin
  • > Penicillin
  • > Cephalosporins
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7
Q

Pemphigus vulgaris

-> HLA associations?

A

-> DR4, DR14, B15, etc.

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8
Q

Pemphigus vegetans

-> Two types?

A
  • > Neumann type, starts and ends like PV

- > Hallopeau type, begins with pustules that quickly turns into malodorous plaques

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9
Q

Pemphigus vegetans

-> Histology?

A
  • > Acantholysis suprabasilar and subcorneal, subtle
  • > Marked hyperplasia and papillomatosis
  • > Eosinophilic abscesses
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10
Q

Pemphigus foliaceus

-> Which sign positive?

A

-> Nikolsky’s sign

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11
Q

Pemphigus foliaceus

-> Histology?

A
  • > Subcorneal or intragranular acantholysis with “cling ons (acantholytic cells)”
  • > Can resemble impetigo with neutrophils in blister cavity
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12
Q

Pemphigus foliaceus

-> DIF pattern?

A

-> Intercellular IgG4, more prominent in upper epidermis

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13
Q

Pemphigus foliaceus

  • > IIF substrate?
  • > Abs?
A
  • > Guinea pig esophagus, intercellular in 80%

- > Desmoglein 1

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14
Q

-> Desmoglein 1 mutation?

A

-> PPK striata type

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15
Q

-> Plakoglobin muatation?

A

-> Naxos syndrome (AR syndrome with right-sided cardiomyopathy, striated PPK, wooly hair)

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16
Q

Paraneoplastic pemphigus

-> Histology?

A
  • > Suprabasilar acantholysis

- > Dyskeratotic keratinocytes with basal vacuolar change

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17
Q

Paraneoplastic pemphigus

-> DIF pattern?

A

-> IgG and C3 intercellular and linear/granular at DE junction

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18
Q

Paraneoplastic pemphigus

  • > IIF substrate?
  • > Abs?
Study These Flashcards
A
  • > Rat bladder, intercellular IgG

- > Plectin, desmoplakin 1 and 2, BPAg1, envoplakin, periplakin, 170 kDa Ag, desmoglein 1 and 3

19
Q

Paraneoplastic pemphigus

-> Malignancy association in order?

Study These Flashcards
A
  • > Non-Hodgkin’s lymphoma (42%)
  • > CLL (29%)
  • > Castleman’s (6%)
  • > Sarcoma (6%)
  • > Thymoma (6%)
20
Q 
Pemphigus erythematosus (Senear-Usher Syndrome)
-> Clinical?
Study These Flashcards
A
  • > Overlap of lupus erythematosus and pemphigus foliaceus

- > Erythematous crusts and hyperkeratotic lesions, occasional bullae on the scalp and face, chest and exts

21
Q

Pemphigus erythematosus

-> Histology?

Study These Flashcards
A

-> Same as pemphigus foliaceus

22
Q

Pemphigus erythematosus

-> DIF pattern?

Study These Flashcards
A
  • > Like pemphigus foliaceus, and with linear IgG at DE junction
  • > Lupus band positive in 80%
  • > ANA positive in 30%
23
Q

Epidermolysis bullosa

-> Clinical?

Study These Flashcards
A
  • > Minor mechanical trauma leads to blisters
  • > Generally non-inflamed, and heals with or without scarring and milia
  • > Nail dystrophy and alopecia can present in all types
24
Q

Four types of epidermolysis bullosa simplex?

Study These Flashcards
A
  • > EB simplex, localized
  • > EB simplex, generalized
  • > EB simplex, Dowling-Meara (herpetiformis)
  • > EB simplex with muscular dystrophy (plectin)
25
Which gene defect in EB simplex?
-> Keratin 5/14
26
Four types of junctional EB and gene defect?
- > Junctional EB, non-Herlitz (BPAg2) - > Junctional EB, Herlitz (laminin 332) - > Junctional EB with pyloric atresia (alpha6-beta-4 integrin) - > EB dystrophic (collagen VII)
27
Epidermolysis Bullosa Acquisita | -> Clinical?
- > Like dystrophic EB, but in adults | - > Trauma-induced bullae (often hands and feet) that heal with scarring and milia, increased skin fragility
28
Epidermolysis Bullosa Acquisita | -> Histology?
-> Subepidermal, classically non-inflammatory (like PCT or dystrophic EB)
29
Epidermolysis Bullosa Acquisita | -> DIF pattern?
- > Linear IgG at BM in 100% | - > Salt-split skin (rather than roof for BP)
30
Epidermolysis Bullosa Acquisita - > IIF pattern? - > Abs?
- > IgG at BM | - > Type VII collagen
31
Cicatricial pemphigoid | -> Clinical?
- > Erosions or ulcers on the oral mucosa - > Desquamative gingivitis - > Conjunctiva
32
Cicatricial pemphigoid - > Histology? - > DIF pattern? - > IIF pattern?
- > Subepithelial bullae, similar to BP - > Linear C3, IgG, fibrinogen at BM in 90% - > Linear at BM in 20%
33
Cicatricial pemphigoid (Abs) - > Mucosal and skin? - > Ocular form? - > Associated with malignancy?
- > BPAg2, distal C-terminal domain - > beta-4-integrin - > laminin 5 (epiligrin, BM600, kalinin, nicein)
34
Drug-induced cicatricial pemphigoid | -> Name drugs
- > Topical medications for glaucoma | - > CV drugs (practolol)
35
Brunsting-Perry pemphigoid
- > No mucosal involvement | - > Blisters on patches of erythema on head/neck with scarring and scarring alopecia
36
Dermatitis herpetiformis | -> Histology?
-> Suprapapillary vesicles, mutilocular, containing mostly neutrophils, papillary dermis and BMZ get destroyed due to inflammatory infiltrate (no festooning)
37
Dermatitis herpetiformis - > DIF pattern? - > IIF pattern?
- > Granular IgA > C3 in dermal papillae in 100% | - > Negative
38
Dermatitis herpetiformis - > Abs? - > Circulating abs in celiac disease?
- > Transglutaminase 3 | - > Anti-endomysial and anti-gliadin antibodies
39
Dermatitis herpetiformis | -> Association?
- > HLA-DQ2 - > Thyroid disease (40%) - > Small bowel lymphoma (non-Hodgkin's lymphoma) - > 90-100% have abnormal jejunal biopsies, but most are asymptomatic in terms of celiac disease
40
Subepidermal split | -> PLAID
- > P: Pemphigoid (bullous) - > L: Lupus (bullous SLE) - > A: Acquisita (EBA) - > I: IgA, linear - > D: Dermatitis herpetiformis
41
Epidermolytic ichthyosis | -> Clinical?
-> Blisters that resolve, subsequently develop "corrugated" layered scaling (generalized but prominent over flexural areas)
42
Epidermolytic ichthyosis | -> Defect in which gene?
-> Keratin 1/10
43
Fogo selvagem - > Etiology? - > Histology? - > Abs?
- > Likely infectious disease transmitted by the black fly (Simulium) - > Identical to pemphigus foliaceus - > Identical to pemphigus foliaceus

Bullous and Vesicular Dermatoses (3 decks)

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