Pemphigus foliaceus is a generally benign blistering disorder. It is characterized by splitting high in the epidermis, causing erosions to form on the skin.

Pemphigus foliaceus usually occurs in middle-aged patients. Foci of high incidence occur in South America, especially Brazil.

The primary lesion is a flaccid bulla. However, because splitting occurs high in the epidermis, bullae are rarely seen; the blisters are so fragile that they rupture. Clinically, scaly, crusted cutaneous erosions, often on an erythematous base, can be seen. Mucosal surfaces are not usually involved. In one variant, pemphigus erythematosus, lesions occur on light-exposed skin and are often similar to those of cutaneous lupus erythematosus.

Diagnosis is by biopsy of a lesion and neighboring normal skin and by serum antibody titers against the cell adhesion molecule desmoglein 1 (160 kd). Because the disorder is much more benign than pemphigus vulgaris, treatment is generally less aggressive. Superpotent topical corticosteroids may be sufficient in some patients. Others require oral prednisone and additional immunosuppressants. A combination of tetracycline 500 mg qid and nicotinamide 1.5 g/day has been effective in some patients. Plasmapheresis is an option for severe disease.

Last full review/revision September 2008 by Julie E. Russak, MD