Article
Hailey-Hailey disease: clinical, diagnostic and therapeutic update.
Anais brasileiros de dermatologia - 1 Jan 2000
Porro Adriana Maria, Arai Seque Camila, Miyamoto Denise, Vanderlei Medeiros da Nóbrega Diego, Simões E Silva Enokihara Milvia Maria, Giuli Santi Claudia
Abstract excerpt
Hailey-Hailey disease is a rare genodermatosis described in 1939, with an autosomal dominant inheritance pattern, characterized by compromised adhesion between epidermal keratinocytes. It has an estimated prevalence of 1/50,000, with no gender or race predilection. It results from a heterozygous mutation in the ATP2C1 gene, which encodes the transmembrane protein hSPA1C, present in all tissues, with preferential...
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