Chapter 52 - Pemphigus Flashcards

1
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

CUTANEOUS FINDINGS
Flaccid blisters, typically sparing palmoplantar surface
Large erosions are common presentations
(+) Nikolsky sign

A

Pemphigus vulgaris

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2
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

CUTANEOUS FINDINGS
Erosions develop excessive papillomatosis tissue and crusting; intertriginous areas, scalp, or face

A

Pemphigus vegetans

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3
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

CUTANEOUS FINDINGS
Scaly, crusted lesions on erythematous base; seborrheic distribution (face, scalp, upper trunk); small flaccid blisters are transient primary lesions

A

Pemphigus foliaceus

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4
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

CUTANEOUS FINDINGS
Crusted lesions in seborrheic distribution

A

Pemphigus erythematosus

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5
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

CUTANEOUS FINDINGS
Similar to pemphigus foliaceus; characterized by burning sensation, and exacerbation on sun exposure

A

Endemic pemphigus foliaceus
(fogo selvagem)

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6
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

Variant of pemphigus vulgaris

A

Pemphigus vegetans

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7
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

Variant of pemphigus foliaceus

A

Pemphigus erythematosus

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8
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

Similar to pemphigus foliaceus clinically, histologically, and serologically, however with distinct epidemiologic features

A

Endemic pemphigus foliaceus
(fogo selvagem)

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9
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

MUCOUS MEMBRANE INVOLVEMENT
Oral and nasal mucous membranes most commonly affected
Esophageal
Vulvar, cervical, vaginal
Ocular

A

Pemphigus vulgaris

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10
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

MUCOUS MEMBRANE INVOLVEMENT
Oral involvement is common

A

Pemphigus vegetans

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11
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

MUCOUS MEMBRANE INVOLVEMENT
Very rare

A

Pemphigus foliaceus

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12
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

MUCOUS MEMBRANE INVOLVEMENT
Rare
(2)

A

Pemphigus erythematosus

Endemic pemphigus foliaceus
(fogo selvagem)

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13
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

PATHOLOGY
Suprabasal blister with acantholysis “row of tombstones” appearance

A

Pemphigus vulgaris

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14
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

PATHOLOGY
Suprabasal acantholysis, with papillomatosis of the dermal papillae and downward growth of epidermal strands into the dermis; presence of hyperkeratosis and scale-crust; eosinophilic or neutrophilic intraepidermal abscesses

A

Pemphigus vegetans

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15
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

PATHOLOGY
Early lesions show eosinophilic spongiosis; histopathology demonstrates acantholysis below stratum corneum; epidermis beneath the granular layer remains intact; subcorneal pustules containing neutrophils and acantholytic epidermal cells in the blister cavity

A

Pemphigus foliaceus

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16
Q

TABLE 52-2
Pemphigus Subtypes and Variants

DIAGNOSIS

PATHOLOGY
Similar to pemphigus foliaceus
(2)

A

Pemphigus erythematosus

Endemic pemphigus foliaceus
(fogo selvagem)

17
Q

TABLE 52-2
Pemphigus Subtypes and Variants

INDIRECT IMMUNOFLUORESCENCE
Ideal substrate for Pemphigus vulgaris

A

Monkey esophagus

18
Q

TABLE 52-2
Pemphigus Subtypes and Variants

INDIRECT IMMUNOFLUORESCENCE
Ideal substrate for Pemphigus foliaceus

A

Guinea pig esophagus or human skin

19
Q

TABLE 52-2
Pemphigus Subtypes and Variants

ELISA
Antibodies in Pemphigus vulgaris with mucosal and skin involvement

A

Desmogleins 1, 3

20
Q

TABLE 52-2
Pemphigus Subtypes and Variants

ELISA
Antibodies in Pemphigus vulgaris with mucosal only involvement

A

Desmoglein 3

21
Q

TABLE 52-2
Pemphigus Subtypes and Variants

ELISA
Antibodies in Pemphigus vegetans

A

Desmoglein 3, sometimes desmoglein 1

22
Q

TABLE 52-2
Pemphigus Subtypes and Variants

ELISA
Antibodies in Pemphigus foliaceus, Pemphigus erythematosus, Endemic pemphigus foliaceus

A

Desmoglein 1