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Hailey-Hailey Disease Is Not Allelic to Darier's Disease

1994; Elsevier BV; Volume: 102; Issue: 6 Linguagem: Inglês

10.1111/1523-1747.ep12384283

1523-1747

Elizabeth A. Welsh, Shigaku Ikeda, Anna Maria Peluso, Jeannette M. Bonifas, John W. Bare, David T. Woodley, Ervin H. Epstein,

Hedgehog Signaling Pathway Studies

Hailey-Hailey (Familial Benign Chronic Pemphigus) Disease is a rare autosomal dominant disorder characterized by blisters caused by suprabasal epidermal acantholysis. Another autosomal dominant skin disease, Darier's disease, has clinical and histologic features which overlap those 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 mutation 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 two kindreds affected with Hailey-Hailey disease.