Chapter 52 - Pemphigus Flashcards
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
CUTANEOUS FINDINGS
Flaccid blisters, typically sparing palmoplantar surface
Large erosions are common presentations
(+) Nikolsky sign
Pemphigus vulgaris
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
CUTANEOUS FINDINGS
Erosions develop excessive papillomatosis tissue and crusting; intertriginous areas, scalp, or face
Pemphigus vegetans
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
Pemphigus foliaceus
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
CUTANEOUS FINDINGS
Crusted lesions in seborrheic distribution
Pemphigus erythematosus
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
CUTANEOUS FINDINGS
Similar to pemphigus foliaceus; characterized by burning sensation, and exacerbation on sun exposure
Endemic pemphigus foliaceus
(fogo selvagem)
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
Variant of pemphigus vulgaris
Pemphigus vegetans
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
Variant of pemphigus foliaceus
Pemphigus erythematosus
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
Similar to pemphigus foliaceus clinically, histologically, and serologically, however with distinct epidemiologic features
Endemic pemphigus foliaceus
(fogo selvagem)
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
MUCOUS MEMBRANE INVOLVEMENT
Oral and nasal mucous membranes most commonly affected
Esophageal
Vulvar, cervical, vaginal
Ocular
Pemphigus vulgaris
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
MUCOUS MEMBRANE INVOLVEMENT
Oral involvement is common
Pemphigus vegetans
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
MUCOUS MEMBRANE INVOLVEMENT
Very rare
Pemphigus foliaceus
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
MUCOUS MEMBRANE INVOLVEMENT
Rare
(2)
Pemphigus erythematosus
Endemic pemphigus foliaceus
(fogo selvagem)
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
PATHOLOGY
Suprabasal blister with acantholysis “row of tombstones” appearance
Pemphigus vulgaris
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
Pemphigus vegetans
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
Pemphigus foliaceus
TABLE 52-2
Pemphigus Subtypes and Variants
DIAGNOSIS
PATHOLOGY
Similar to pemphigus foliaceus
(2)
Pemphigus erythematosus
Endemic pemphigus foliaceus
(fogo selvagem)
TABLE 52-2
Pemphigus Subtypes and Variants
INDIRECT IMMUNOFLUORESCENCE
Ideal substrate for Pemphigus vulgaris
Monkey esophagus
TABLE 52-2
Pemphigus Subtypes and Variants
INDIRECT IMMUNOFLUORESCENCE
Ideal substrate for Pemphigus foliaceus
Guinea pig esophagus or human skin
TABLE 52-2
Pemphigus Subtypes and Variants
ELISA
Antibodies in Pemphigus vulgaris with mucosal and skin involvement
Desmogleins 1, 3
TABLE 52-2
Pemphigus Subtypes and Variants
ELISA
Antibodies in Pemphigus vulgaris with mucosal only involvement
Desmoglein 3
TABLE 52-2
Pemphigus Subtypes and Variants
ELISA
Antibodies in Pemphigus vegetans
Desmoglein 3, sometimes desmoglein 1
TABLE 52-2
Pemphigus Subtypes and Variants
ELISA
Antibodies in Pemphigus foliaceus, Pemphigus erythematosus, Endemic pemphigus foliaceus
Desmoglein 1