Atypical features in familial benign chronic pemphigus.

Abstract

As the clinical concept and histologic specificity of familial benign chronic pemphigus have gradually become widely accepted, this disease entity has been recognized with increased frequency. Over 110 confirmed cases have appeared in the literature since the original description by Hailey and Hailey in 1939.¹Our interest in writing this report was stimulated by the recent finding of six atypical cases of familial benign chronic pemphigus. It is our purpose to call attention to the unusual features of these cases. During the process of academic recognition and acceptance, familial benign chronic pemphigus has received a variety of descriptive names, such as chronic recurrent acantholysis (Pinkus and Epstein),²vesicular Darier's disease (Ellis),³recurrent herpetiform dermatitis repens (Ayears and Anderson),⁴dyskeratosis bullosa hereditaria (Becker and Obermayer),⁵and dyskeratoid dermatosis (Frank and Rein).⁶There is agreement, however,