Hailey-Hailey (Familial Benign Chronic Pemphigus) Disease is a rare au
tosomal dominant disorder characterized by blisters caused by suprabas
al epidermal acantholysis. Another autosomal dominant skin disease, Da
rier's disease, has clinical and histologic features which overlap tho
se of Hailey-Hailey disease and recently has been mapped to chromosome
12q23-q24.1. We have used linkage analysis to test whether or not a m
utation in this region might also underlie Hailey-Hailey disease. This
analysis, using polymorphic loci tightly linked to Darier's disease,
excluded this region as the site for the disease-causing mutation in t
wo kindreds affected with Hailey-Hailey disease.