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Article
May 1950

CHRONIC BENIGN FAMILIAL PEMPHIGUS, A PROBABLE VESICULAR VARIANT OF KERATOSIS FOLLICULARIS

Arch Derm Syphilol. 1950;61(5):737-749. doi:10.1001/archderm.1950.01530120028002
Abstract

THE PROBLEM concerning the exact nosologic position of chronic familial benign pemphigus has been a matter of conjecture and a subject for lively debate ever since this malady was originally described in 1939 by Hailey and Hailey.1 Its similarity, clinically or histologically, to pemphigus, Darier's disease, dermatitis repens, epidermolysis bullosa and dermatitis herpetiformis has brought forth a large number of case reports and articles concerning its probable correct classification. As yet this problem is unsolved. Some believe that it is a distinct entity in itself. We believe that so-called chronic familial benign pemphigus is a vesicular variant of Darier's disease.

Hailey and Hailey described 4 cases which resembled impetigo. The history of recurrences of the bullae, the familial tendency and the confusing histologic picture which resembled both pemphigus and keratosis follicularis were considered as pointing to a benign form of pemphigus. Finally, they concluded that the

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