What is Pemphigus Vulgaris?

Pemphigus Vulgaris is a rare disease that affects elderly patients. The mortality rate is 10%; most deaths result from steroid complications, secondary infection, dehydration, or thromboembolism. Pemphigus Vulgaris is caused by the attachment of immunoglobulin G autoantibodies to the epidermis. It has been associated with D -penicillamine and captopril administration.

CLINICAL FEATURES

1. Mucosal lesions and erosions are very common. Examination of all mucosal sites is warranted.

2. Non-pruritic, painful, flaccid bullae appear that rupture easily. Blisters can be extended or new bullae formed by applying firm tangential pressure on the intact epidermis.

3. Weakness, weight loss, and dysphagia may be presenting complaints.

DIFFERENTIAL DIAGNOSES

— Erythema multiforme

— Bullous impetigo

— Herpes zoster

EVALUATION: Biopsy of lesions shows eosinophils, intraepidermal bullae, and acantholysis. Indirect immunofluorescent staining shows immunoglobulin G antibodies. Serum titers can be followed to evaluate the effectiveness of therapy.

THERAPY

1. Prednisone (200 to 350 mg/day) for 5 to 10 weeks is used until the cessation of new blister formation occurs. The dosage is then reduced to 40 mg on alternative days and tapered over 1 year.

2. Azathioprine (100 mg/day) is added to the regimen and the dosage is reduced over a 4- to 6-month period. Methotrexate and cyclophosphamide can be used instead of azathioprine.

3. Topical analgesics (e.g., viscous lidocaine) can be used to alleviate the pain associated with oral lesions.

DISPOSITION: Patients with severe cases and oral lesions may require hospital admission for intravenous hydration. Others can be treated as outpatients with close follow-up.