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Pemphigus Foliaceus

By Daniel M. Peraza, MD, The Geisel School of Medicine at Dartmouth

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Patient Education

Pemphigus foliaceus is an autoimmune blistering disorder in which splits in the superficial epidermis result in cutaneous erosions.

Pemphigus foliaceus usually occurs in middle-aged patients, affecting men and women in equal numbers. An endemic form of pemphigus foliaceus, fogo selvagem, occurs in younger adults and children, particularly in South America. Pemphigus erythematosus, a localized form of pemphigus foliaceus, has immunologic features of pemphigus and lupus erythematosus (IgG and C3 deposition on keratinocyte surfaces and basement membrane zone with circulating antinuclear antibodies); however, patients rarely are diagnosed with both diseases concurrently. Pemphigus foliaceus may occur after use of penicillamine, nifedipine, or captopril.

Symptoms and Signs

The primary lesion is a flaccid vesicle or bulla, but due to the superficial location of the epidermal split, lesions tend to rupture, so intact bullae or vesicles are rarely evident on examination. Instead, well-demarcated, scattered, crusted, erythematous lesions are common on the face, scalp, and upper trunk. Mucosal involvement is rare. Skin lesions can burn and cause pain, but patients are typically not severely ill. Pemphigus erythematosus tends to affect the malar cheeks.

Diagnosis

  • Skin biopsy and immunofluorescence

Diagnosis of pemphigus foliaceus is by biopsy of a lesion and adjacent (perilesional) unaffected skin that shows IgG autoantibodies against the keratinocyte cell surface via direct immunofluorescence. Autoantibodies to desmoglein 1, a transmembrane glycoprotein that affects cell-cell adhesion and signaling between epidermal cells, can be detected in serum via direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA).

Treatment

  • Corticosteroids, topical or systemic

If the disease is localized and not severe, high-potency topical corticosteroids are typically effective. More widespread or severe cases require systemic corticosteroids plus, at times, other immunosuppressive therapies, such as rituximab, plasma exchange, methotrexate, mycophenolate mofetil, or azathioprine. Limited studies suggest that addition of a combination of tetracycline 500 mg po qid and nicotinamide 500 mg po tid may be effective in some people.

* This is the Professional Version. *