-
Incidence: 0.5 to 3.2 cases per 100,000 (U.S.)
- Higher Incidence in Ashkenazi jews
- Mean age of onset: 40 to 60 years old
- Men and women are affected equally
- Most common, severe and deeper form of Pemphigus
- Ashkenazi jewish descent
-
Autoimmune Condition (e.g. SLE, Rheumatoid Arthritis, Myasthenia Gravis)
- Triggers
- See medications below
- Burn Injury
- Infections
- IgG against Keratinocyte cell surface molecules (desmoglein 1 and 3) in skin and mucosa
- Antibody binding results in Acantholysis (loss of cell to cell adhesion)
- May be unmasked by certain medications (e.g. Penicillin, NSAIDs, ACE Inhibitors, Pyrazolones)
- See Drug-Triggered Pemphigus
- Painful, often burning lesions (may be pruritic)
- Oral symptoms with mucosal involvement
- Dysphagia
- Hoarseness
- Epistaxis
- Constitutional symptoms
- Weakness
- Malaise
- Mucosal sites of involvement
- Painful Gingival erosions (50-70% of patients)
- May precede skin bullae by months
- Conjunctiva and Esophagus may also be involved
- Flaccid bullae (difficult to see due to flaccidity)
- Clear fluid filled Blisters with thin walls that easily rupture, developing into painful erosions
- Blisters overly an erythematous base
- Nikolsky Sign positive
- Painful erosions (most common skin finding)
- May bleed easily
- Crusting is often present
- Lesions are painful rather than pruritic (contrast with Bullous Pemphigoid)
- Skin Sites of involvement
- Face
- Scalp
- Upper body
- Intertriginous areas (axillae, groin)
- Umbilicus
- Nails (Subungual Hematoma, Chronic Paronychia)
- Biopsy of bulla margin
- Suprabasilar Blister (above Basal Cell Layer)
- Acantholysis
- Rounded basal cells appear as row of tombstones
- Eosinophilic infiltrates
- Direct Immunofluorescence
- Intercellular deposits of IgG and C3 on Keratinocytes
- Onset on Oral Mucosa
- Skin lesions follow Oral Lesions by months
- Localized skin involvement for 6-12 months
-
Generalized involvement then ensues
- Thymoma (and Myasthenia Gravis)
- Possible complications of immunosuppressive therapy
- Kaposi's Sarcoma
- Lymphoreticular malignancy
- Management
-
Immunosuppressive Therapy
- Disposition
- Most patients are treated out of the hospital
- Admit patients with extensive bullae and erosions for IV fluids and Electrolyte management
-
Prednisone 1 mg/kg/day
- Reduce dose by 50% when no new Blister formation
- Gradually taper to minimum effective dose
- Topical Corticosteroids may also be used as an adjunct
- Adjunctive Immunosuppressive Drugs
- Methotrexate
- Azathioprine (Imuran)
- Cyclophosphamide (Cytoxan)
- Mycophenolate Mofetil (Cellcept)
- Other measures in severe cases
- Plasmapheresis
- Secondary infection (due to immunosuppressive therapy)
- Mortality highest in first few years (up to 10%)
- Complications of Corticosteroids
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