II. Epidemiology
- Incidence (rare): 1 in 12,000 to 20,000
- Ages affected: 60 to 80 years
- Gender predominence: Women by ratio of 2:1
III. Pathophysiology
- Autoimmune Bullous Condition
- Associated conditions
- Stevens-Johnson Syndrome
- Medications
IV. Signs
-
Blistering of mucus membranes
- Blister ruptures within hours of formation
- Painful erosion
- Most erosions heal without scarring
- Most common sites of involvement
- Oral Mucosa (most often buccal and palatal mucosa)
- Results in erosive Gingivitis
- Conjunctiva (usually bilateral)
- Results in chronic Conjunctivitis and scarring
- Oral Mucosa (most often buccal and palatal mucosa)
- Other sites of involvement
V. Labs: Histology
- Subepidermal bulla
- Perivascular inflammatory cell infiltrates
- Fibrosis distinguishes from Bullous Pemphigoid
- Immunofluorescence with IgG, C3, IgA deposits
- Forms linear band at Basement Membrane Zone
VI. Differential Diagnosis
- See Autoimmune Bullous Condition
- Bullous Pemphigoid
- Epidermolysis Bullosa Acquisita (EBA)
- Linear IgA Bullous Dermatosis (LABD)
- Systemic Lupus Erythematosus
- Pseudopemphigoid
- Pemphigus
VII. Management: Early aggressive treatment
- First-Line: Prednisone 0.5 to 0.75 mg/kg/day x6 months
- Ocular Involvement: Azathioprine or Cyclophosphamide
- Skin Involvement: Topical Corticosteroids
- Oral Involvement
- Dapsone
- Corticosteroid gel
- Dexamethasone mouthwash (Roxane)
VIII. Complications
- Ocular Lesions may result in blindness
- Laryngeal stricture
- Esophageal Stricture
- Supraglottic Stenosis