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Grand Rounds
August 11, 1999

Pemphigus—Diseases of Antidesmosomal Autoimmunity

Author Affiliations

Author Affiliations: Dermatology Branch, Division of Clinical Sciences, National Cancer Institute, Bethesda, Md (Dr Udey); and the Department of Dermatology, University of Pennsylvania, Philadelphia (Dr Stanley).


Grand Rounds at the Clinical Center of the National Institutes of Health Section Editors: John I. Gallin, MD, the Clinical Center of the National Institutes of Health, Bethesda, Md; David S. Cooper, MD, Contributing Editor, JAMA.

JAMA. 1999;282(6):572-576. doi:10.1001/jama.282.6.572

A 78-year-old white man was referred to the Dermatology Branch of the National Cancer Institute (NCI), Bethesda, Md, with a 5-month history of a bullous disorder. The patient initially went to a community dermatologist and presented with gingival and buccal erosions and with occasional flaccid blisters, crusted papules, and plaques involving the scalp, back, and chest. A provisional diagnosis of pemphigus vulgaris (PV) was made, and although biopsy results were nondiagnostic, indirect immunofluorescence testing revealed deposits of IgG on the surfaces of keratinocytes in monkey esophagus. He initially received prednisone (60 mg/d) and tetracycline hydrochloride (500 mg 4 times per day), but the regimen was changed to prednisone (100 mg/d) and azathioprine (100 mg/d) 2 weeks later because of an inadequate clinical response. The patient's disease remained active over the next 4 months despite treatment with prednisone (≥60 mg/d) and azathioprine (150 mg/d), and the patient was referred for evaluation.