Pemphigus
Epidemiology:
- 30s to 50s
- pemphigus vulgaris
is the most common (80%)
- pemphigus
foliaceous occus in epidemic form in South America
Common sites:
- pemphigus
vulgaris:
- mucosa (may be
first site of involvement)
- scalp
- face
- axilla
- groin
- trunk
- pressure points
- pemphigus
vegetans:
- groin
- axillae
- flexural
surfaces
- pemphigus
foliaceus:
- pemphigus
erythematosus:
Gross features:
- pemphigus
vulgaris:
- oral ulcers
- superficial
vesicles or bullae that rupture easily
- shallow
erosions covered with dried serum and crust
- pemphigus
vegetans:
- not blisters
- large, moist,
verrucous, vegetating plaques studded with pustules
- pemphigus
foliaceus:
- zones of
erythema and cursting (bullae are very superficial and rupture easily)
Histologic features:
- acantholysis
- dissolution of
intercellular junctions
- keratinocytes
lose their polyhedral shape and become rounded
- variable
superficial dermal infiltrate:
- lymphocytes,
histiocytes, eosinophils
- pemphigus
vulgaris:
- acantholysis of
the layer of cells immediately above the basal cell layer
- basal layer
looks like a row of tombstones
- pemphigus
vegetans:
- acantholysis of
the layer of cells immediately above the basal cell layer
- considerable
overlying acanthosis
- pemphigus foliaceus:
- acantholysis of
the stratum granulosum
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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- net-like
pattern of intercellular IgG deposits localized to sites of acantholysis
Molecular features:
Other features:
- autoimmune
- Serum IgG to
intercellular cement substance of skin and mucous membranes
- desmoglein
3 is antigen in pemphigus vulgaris
- desmoglein
1 in pemphigus foliaceus
- may be
life-threatening without treatment
References: