Pemphigus Vulgaris

1. See Also
   1. Bullous Disease
   2. Pemphigus
2. Epidemiology
   1. Incidence: 0.1 to 0.5 cases per 100,000 (worldwide)
   2. Higher Incidence in Ashkenazi jews
   3. Mean age of onset: 40 to 60 years old
3. Definition
   1. Most common, severe and deeper form of Pemphigus
4. Pathophysiology
   1. IgG against desmoglein 3 in skin and mucosa
   2. May be unmasked by certain medications
      1. See Drug-Triggered Pemphigus
5. Symptoms
   1. Painful, often burning lesions (may be pruritic)
   2. Oral symptoms with mucosal involvement
      1. Dysphagia
      2. Hoarseness
      3. Epistaxis
   3. Constitutional symptoms
      1. Weakness
      2. Malaise
6. Signs
   1. Mucosal sites of involvement
      1. Painful Gingival Erosions (50-70% of patients)
      2. May precede skin bullae by months
   2. Flaccid bullae (difficult to see due to flaccidity)
      1. Easily rupture, developing into painful Erosions
      2. Nikolsky's Sign positive
   3. Painful Erosions (most common skin finding)
      1. May bleed easily
      2. Crusting is often present
   4. Skin Sites of involvement
      1. Face
      2. Scalp
      3. Upper body
      4. Intertriginous areas (axillae, groin)
      5. Umbilicus
7. Labs
   1. Biopsy of bulla margin
      1. Suprabasilar Blister (above Basal Cell Layer)
      2. Acantholysis
      3. Rounded basal cells appear as row of tombstones
   2. Direct Immunofluorescence
      1. Intercellular deposits of IgG and C3
8. Course
   1. Onset on Oral Mucosa
   2. Skin lesions follow Oral Lesions by months
   3. Localized skin involvement for 6-12 months
   4. Generalized involvement then ensues
9. Associated Conditions
   1. Thymoma (and Myasthenia Gravis)
   2. Possible complications of immunosuppressive therapy
      1. Kaposi's Sarcoma
      2. Lymphoreticular malignancy
10. Variants
    1. Pemphigus Vegetans (Familial Benign Pemphigus)
11. Management: Immunosuppressive Therapy
    1. Prednisone 1 mg/kg/day
       1. Reduce dose by 50% when no new Blister formation
       2. Gradually taper to minimum effective dose
    2. Adjunctive immunosuppressive drugs
       1. Methotrexate
       2. Azathioprine (Imuran)
       3. Cyclophosphamide (Cytoxan)
       4. Mycophenolate mofetil (CellCept)
    3. Other measures in severe cases
       1. Plasmapheresis
12. Complications
    1. Secondary infection (due to immunosuppressive therapy)
13. Prognosis
    1. Mortality highest in first few years (up to 10%)
       1. Complications of Corticosteroids
14. References
    1. Cotran (1999) Robbins Pathology, p. 1202
    2. Bickle (2002) Am Fam Physician 65(9):1861-70
    3. Cotell (2000) Am J Emerg Med 18(3):288-99
    4. Rye (1997) Am Fam Physician 55(8): 2709-18