Pemphigus

Pemphigus 

Updated: 02/09/2021

© Jun Wang, MD, PhD

General features

• Rare, nonhereditary, chronic, autoimmune disease
• Ages 30-59; no gender preference
• May be associated with internal malignancies, including thymoma
• Intraepidermal blisters with acantholysis
• Benign, but may be fatal if untreated in extreme cases

Pathogenesis

• Autoantibody against desmoglein 1 and 3 (desmosome components)

Pemphigus vulgaris

• Most common type
• May involve both skin and oral mucosa
• Superficial vesicles, easily ruptured with painful erosion
• Healing usually accompanied by post-inflammatory hyperpigmentation
• Deaths may occur due to staphylococcal infection, or pulmonary embolism
• Suprabasilar bullae with single row of basal cells resides on basement membrane

Pemphigus foliaceus

• Endemic in Brazil and Columbia, but may be sporadic in the world
• Often involving skin of trunk
• Mucosa NOT involved
• Crusts and shallow erosions on healthy skin with blisters formed after rubbing
• Mild clinical behavior
• Vesicles at the granular layer with mild inflammation

Pemphigus erythematosus

• Senear–Usher syndrome
• Usually middle age adults
• Localized area, resemble lupus malar rash on face
• May be considered as a localized less severe form of pemphigus foliaceus

Pemphigus vegetans

• Rare
• Considered a localized form of pemphigus vulgaris
• Verrucous plaques in affected regions
• Oral cavity commonly affected
• Subtle supra basal acantholysis with epidermal hyperplasia, intraepidermal eosinophilic and neutrophilic microabscesses

Pemphigus paraneoplastic

• Associated with internal tumor
• Involving both oral mucosa and skin

Pathological features

• Intraepidermal vesicles
• Acantholysis
  
• May have eosinophilic infiltrate

Immunofluorescence feature

• IgG along intercellular junctions within epidermis

Management

• Corticosteroid
• Immunosuppressant
• Wound care, etc

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