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pemphigus vulgaris blisters acantholysis histology
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Pemphigus — Autoimmune Blistering Disease
Note: "Pampigus Diesese" = Pemphigus (from Greek pemphix = blister/bubble)
What Is Pemphigus?
Pemphigus is a group of chronic autoimmune blistering diseases of the skin and mucous membranes. It is characterized by:
- Intraepidermal blisters due to acantholysis (loss of cell-to-cell adhesion between keratinocytes)
- IgG autoantibodies targeting desmogleins — transmembrane desmosomal adhesion proteins
- The antibodies disrupt intercellular adhesion and may activate intercellular proteases
It was first understood as an autoimmune disease in 1964 when Ernst Beutner and Robert Jordon discovered circulating anti-keratinocyte antibodies in pemphigus vulgaris.
Types / Classification
| Subtype | Autoantibody Target | Level of Blister | Clinical Features |
|---|---|---|---|
| Pemphigus Vulgaris (PV) | DSG1 + DSG3 | Suprabasal | Mucosal + skin erosions; flaccid bullae |
| Pemphigus Foliaceus (PF) | DSG1 only | Subcorneal (stratum granulosum) | Skin only; crusted, scaly lesions; no mucosal involvement |
| Pemphigus Vegetans | DSG1 + DSG3 | Suprabasal | Localized variant of PV; verrucous plaques in flexures |
| Pemphigus Erythematosus | DSG1 | Subcorneal | Localized variant of PF; seborrheic/lupus-like distribution |
| Fogo Selvagem (Endemic PF) | DSG1 | Subcorneal | Endemic in Brazil; triggered by insect bites |
| IgA Pemphigus | Desmocollin 1 | Variable | IgA deposits; less severe |
| Paraneoplastic Pemphigus | Multiple (desmoplakin, etc.) | Suprabasal | Associated with internal malignancy; severe mucosal ulceration |
| Drug-Induced Pemphigus | DSG1 or DSG3 | Variable | ACE inhibitors (captopril), penicillamine, gold |
Pathogenesis
- Autoantibodies (IgG) target desmoglein-1 (DSG1) and/or desmoglein-3 (DSG3) — critical components of desmosomes
- Antibody binding → disruption of intercellular adhesion → acantholysis (cells separate from one another)
- Blister location depends on where desmoglein is expressed:
- DSG3 predominates in mucosa and deeper epidermis → suprabasal blisters in PV
- DSG1 predominates in superficial epidermis → subcorneal blisters in PF
- Associated with specific HLA alleles (genetic predisposition)
- Complement activation also contributes to tissue damage
Clinical Features
Pemphigus Vulgaris (most common)
- Demographics: Older adults; more common in women; higher incidence in Ashkenazi Jews and people of Mediterranean descent
- Sites: Oropharyngeal mucosa (almost always involved first), scalp, face, axillae, groin, trunk, pressure points
- Lesions: Fragile, flaccid bullae that rupture easily → painful, extensive erosions covered with serum crust
- Nikolsky sign: Positive — lateral pressure on normal-looking skin causes epidermal separation
- Lesions are painful and prone to secondary infection
Pemphigus Foliaceus
- Milder form; no mucosal involvement
- Crusted, scaly lesions; blisters are superficial and erythematous
- Widespread in fogo selvagem (endemic variant in Brazil)
Histology
| Feature | Pemphigus Vulgaris | Pemphigus Foliaceus |
|---|---|---|
| Acantholysis level | Suprabasal | Subcorneal (stratum granulosum) |
| Basal cells | "Tombstone row" — remain attached to basement membrane | Detached superficially |
| Blister type | Intraepidermal, suprabasal | Subcorneal |
Immunofluorescence
- Direct IF (DIF): Fishnet / chicken-wire pattern of intercellular IgG and C3 deposits throughout the epidermis — pathognomonic
- Indirect IF: Circulating anti-desmosomal IgG in serum
- ELISA: Anti-DSG1 and anti-DSG3 antibody titers — useful for diagnosis and monitoring disease activity
Diagnosis
- Clinical — flaccid blisters + mucosal erosions + positive Nikolsky sign
- Biopsy — intraepidermal acantholysis
- Direct immunofluorescence — fishnet IgG intercellular pattern
- ELISA — anti-DSG antibody titers
- Indirect immunofluorescence — circulating antibodies on monkey esophagus substrate
Treatment
| Approach | Agents |
|---|---|
| First-line | Systemic corticosteroids (prednisone) |
| Steroid-sparing | Azathioprine, mycophenolate mofetil, cyclophosphamide |
| Biologic | Rituximab (anti-CD20 monoclonal antibody) — now considered first-line in moderate-severe PV |
| Adjuncts | IV immunoglobulin (IVIG), plasmapheresis |
| Topical | Topical corticosteroids for localized lesions |
| Long-term | Many patients require prolonged immunosuppression |
Rituximab has significantly improved outcomes — it depletes B cells and reduces autoantibody production.
Key Comparisons: Pemphigus vs. Bullous Pemphigoid
| Feature | Pemphigus | Bullous Pemphigoid |
|---|---|---|
| Blister location | Intraepidermal | Subepidermal |
| Blister quality | Flaccid, fragile | Tense, firm |
| Nikolsky sign | Positive | Negative |
| Mucosal involvement | Common (especially PV) | Uncommon |
| Autoantibody target | Desmoglein 1/3 (desmosomes) | BP180/BP230 (hemidesmosomes/BMZ) |
| IF pattern | Fishnet intercellular | Linear at BMZ |
| Age group | Middle-aged to older adults | Elderly |
Sources: Fitzpatrick's Dermatology, Vol. 1–2, p. 939 | Robbins & Kumar Basic Pathology, p. ~22 | Dermatology 2-Volume Set, 5e
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