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October 1982

Commentary: Hailey-Hailey Disease: Familial Benign Chronic Pemphigus

Author Affiliations

From the Department of Dermatology, Case Western Reserve University School of Medicine, University Circle, Cleveland.

Arch Dermatol. 1982;118(10):781-783. doi:10.1001/archderm.1982.01650220085010

In April 1939, Howard Hailey and Hugh Hailey,1 from the Department of Dermatology of Emory University School of Medicine, Atlanta, wrote an article in which they described two brothers, aged 35 and 38 years, with recurrent dermatoses of nine and ten years, respectively. It was a problem to classify the conditions of these patients from the onset.

Clinically, the condition was characterized by recurrent small blisters of the neck that became wet and crusted within a few days and spread rapidly. The lesions were generally sharply marginated and varied in size from several millimeters to several centimeters. They had a peripheral scale, and Nikolsky's sign was positive. The lesions healed without scarring, but residual hyperpigmentation was observed. A culture from the lesions yielded short-chain streptococci.

The lesions were pruritic and unilateral or bilateral. The patients thought that "perspiration in the summer added to their discomfort." The condition had been

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