Pemphigus Foliaceus, Pemphigus Erythematosus

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Overview

Pemphigus foliaceus is a relatively benign autoimmune bullous disease associated with the deposition of intercellular antibodies directed against desmoglien 1. The therapeutic approach differs significantly from that for pemphigus vulgaris; therapy is adjusted on the basis of clinical response rather than immunopathologic data. The goal is to control lesion formation, although an occasional blister should be tolerated, as chronic effects of therapy may be more dangerous than the disease. Pemphigus erythematosus may be treated like pemphigus foliaceus.

First Steps, Limited Disease

1. Apply a superpotent topical steroid cream twice daily for 2-3 weeks, supplemented by intralesional injections of triamcinolone acetonide 5 mg/ml to select lesions.
2. Maximum Sun Protection is recommended.

First Steps, Widespread Lesions

1. Continue administration of superpotent topical steroid.
2. Prednisone 20-40 mg/day may be used for severe flares, but chronic therapy should be avoided except in the most severe cases.
3. Dapsone 100-300 mg daily alone or in combination with systemic steroids may be effective in refractory cases, and should be considered as a part of a maintenance regimen.
4. Tetracycline 500 mg 4 times daily or doxycycline 100 mg twice daily, plus nicotinamide 500 mg 3 times daily may be considered as a steroid-sparing regimen, but response is variable.

Alternative Steps

1. If the condition is severe and refractory to the above treatments, consider adding an immunosuppressive agent, either mycophenolate mofetil 2.0-3.0 g/day, or azathioprine 100 mg/day.

Pitfalls

1. Drugs such as penicillamine and captopril may cause pemphigus foliaceus. These should be discontinued. Most drug-induced cases can be treated with superpotent topical steroids.
2. Pemphigus foliaceus may be associated with lupus erythematosus, but rarely. This disorder is called pemphigus erythematosus, and is treated like pemphigus foliaceus.

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