Pemphigus Foliaceus, Pemphigus Erythematosus
Wednesday, February 11, 2009
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
- 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.
- Maximum sun protection is recommended.
First Steps, Widespread Lesions
- Continue administration of superpotent topical steroid.
- Prednisone 20-40 mg/day may be used for severe flares, but
chronic therapy should be avoided except in the most severe
- Dapsone 100-300 mg daily alone or in combination with systemic
steroids may be effective in refractory cases, and should be
considered as part of a maintenance regimen.
- 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.
- 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
- 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.
- Pemphigus foliaceus may be associated with lupus erythematosus,
but rarely. This disorder is called pemphigus erythematosus, and is
treated like pemphigus foliaceus.