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Book - Elston > Ch. 9 - Blistering diseases > Flashcards

Ch. 9 - Blistering diseases Flashcards

1
Q

What are the subcorneal vesiculbullous disorders?

A

Pemphigus foliaceus

Subcorneeal pustular dermatosis

Acute generalized exanthematous pustulosis

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2
Q

Pemphigus foliaceus

(cause?)

A

Subcorneal split with some acantholysis and dyskeratosis. Net-like IgG/C3 in upper epidermis.

(IgG directed at desmoglein 1)

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3
Q

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease)

A

Subcorneal pustule “sitting” upon epidermis. No dyskeratosis. No immunofluorescence.

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4
Q

Acute generalized exanthematous pustulosis

(cause?)

A

Subcorneal epidermal pustules with mild spongiosis and occasional eosinophils. No immunofluorescence.

(reaction to antibiotics)

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5
Q

What are the intraepidermal vesiculbullous disorders?

A

Pemphigus vulgaris

Familial benign chronic pemphigus (Hailey-Hailey disease)

Keratosis follicularis (Darier’s disease)

Transient acantholytic dermatosis (Grover’s disease)

Paraneoplastic pemphigus

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6
Q

Pemphigus vulgaris

(cause?)

A

Basal layer split with follicular extension. Eosinophils. DIF with IgG/C3 between keratinocytes in lower epidermis.

(IgG against desmoglein 3)

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7
Q

Familial benign chronic pemphigus (Hailey-Hailey disease)

(cause?)

A

Acanthotic and acantholytic lesion. Red dyskeratotic rim around nucleus. No immunofluorescence.

(inherited mutation in ATP2C1)

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8
Q

Keratosis follicularis (Darier’s disease)

(cause?)

A

Acantholytic lesion with more dyskeratosis than Hailey-Hailey disease. Grains, corps ronds, blue/clear perinuclear rim, and no immunofluorescence.

(inherited mutation in ATP2A2)

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9
Q

Transient acantholytic dermatosis (Grover’s disease)

A

Many patterns, including Darier’s, Hailey-Hailey’s, Pemphigus, and Spongiotic. May or may not be transient. Not inherited.

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10
Q

Paraneoplastic pemphigus

(cause?)

A

Variable intraepidermal acantholysis with lichenoid infiltration. DIF with IgG/C3 linear deposition at junction.

(many possible antibodies)

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11
Q

What are the pauci-inflammatory subepidermal vesiculbullous disorders?

A

Porphyria cutanea tarda

Epidermolysis bullosa acquisita

TEN/SJS

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12
Q

Porphyria cutanea tarda

A

Subepidermal vesiculation with solar elastosis, festooning of dermal papillae into vesicle, and caterpillar bodies in the epidermis. DIF with IgM/C3 in vessels.

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13
Q

Epidermolysis bullosa acquisita

(cause?)

A

Subepidermal vesiculation with fibrin deposition in floor and inflammatory infiltrate. DIF with IgG/C3 at junction, sometimes IgA.

(antibody to type VII collagen)

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14
Q

TEN/SJS

A

Subepidermal vesiculation and sloughing with confluent epidermal necrosis. Some inflammatory infiltrate. No immunofluorescence.

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15
Q

What are the inflammatory subepidermal vesiculbullous diseases?

A

Bullous pemphigoid

Cicatricial pemphigoid

Dermatitis herpetiformis

Linear IgA bullous dermatosis

Bullous lupus erythematosis

Friction blister

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16
Q

Bullous pemphigoid

(cause?)

A

Subepidermal bulla with eosinophils or neutrophils. DIF with IgG/C3 linearly along junction.

(autoantibodies to bullous pemphigoid antigens 1/2)

17
Q

Cicatricial pemphigoid

A

Subepidermal vesiculation with variable neutrophilic inflammation and scarring. DIF with IgG/C3 in linear junctional pattern.

18
Q

Dermatititis herpetiformis

(cause?)

A

Subepidermal vesiculation with neutrophillic abscesses and fibrin deposition at tips of dermal papillae. DIF with IgA in granular pattern along junction (“picket fence”)

(Deposition of transglutaminase-3 in celiac sprue)

19
Q

Linear IgA bullous dermatosis

A

Subepidermal vesiculation with neutrophils or eosinophils. DIF with IgA in linear pattern along junction.

20
Q

Bullous lupus erythematosis

A

Subepidermal bulla with neutrophils. “Immunoreactants on floor of salt-split skin”?

21
Q

Friction blister

A

Subepidermal bulla with mid-epidermal necrosis and serum.

22
Q

Reticular IgG in upper epidermis

A

Pemphigus foliaceus

23
Q
A

Subcorneal pustular dermatosis

24
Q
A

Acute generalized exanthematous pustulosis (AGEP)

25
Q

Reticular IgG in lower epidermis

A

Pemphigus vulgaris

26
Q
A

Familial benign chronic pemphigus (Hailey-Hailey disease)

27
Q
A

Keratosis follicularis (Darier’s disease)

28
Q
A

Paraneoplastic pemphigus

29
Q
A

Porphyria cutanea tarda

30
Q

Linear serrated IgG at junction

A

Epidermolysis bullosa acquisita

31
Q
32
Q

Linear IgG at junction

A

Bullous pemphigoid

33
Q
A

Cicatricial pemphigoid

34
Q

Granular IgA at junction

A

Dermatitis herpetiformis

35
Q

Linear IgA at junction

A

Linear IgA bullous dermatosis

Book - Elston (30 decks)

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