Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:18

1150 Pemphigus Vulgaris and Pemphigus Foliaceus

■Biopsy of the oral mucosa demonstrates similar findings.

■DIF – intercellular IgG and C3

■IIF – circulating IgG that binds to cell surface of keratinocytes

■ELISA for antibodies to desmoglein 1 and or 3 may be predictive of

clinical activity (desmoglein 3 is associated with pemphigus vulgaris

and desmoglein 1 with pemphigus foliaceus).

differential diagnosis

■Impetigo: Does not cause mucosal erosions

■Bullous pemphigoid: BP has tense as opposed to flaccid bullae

■Erythema multiforme: Negative immunofluorescence studies

management

What to Do First

■Correct fluid and electrolyte imbalances.

General Measures

■Antibiotics for bacterial infection, cleansing baths, wet dressings,

pain management

specific therapy

■Mild/localized disease can be treated with topical and intralesional

corticosteroids along with dapsone or a tetracycline.

■Generalized PV – use systemic corticosteroids along with a steroid-

sparing agent

■Prednisone as a single daily dose, 50% respond. May require divided-

dose corticosteroids.

■Steroid-sparing agents:

➣dapsone

➣minocycline

➣azathioprine

➣mycophenolate mofetil

➣cyclophosphamide

➣methotrexate

➣Intravenous immune globulin

➣Rituximab

■Side Effects & Contraindications

➣Prednisone, azathioprine, mycophenolate mofetil, cyclophos-

phamide, methotrexate, dapsone, rituximab, tetracyclines – see

Specific Therapy for Bullous Pemphigoid