2020
DOI: 10.1111/dth.14429
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Abstract: Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis with complete penetrance that affects the ATP2C1 gene, located in the long arm of chromosome 3 and responsible for encoding the calcium ATPase enzyme of the Golgi complex. Defective calcium channels in keratinocytes impair the formation of desmosome adhesion proteins and lead to acantholysis. 1-3 The estimated incidence is 1/50000 individuals, 3 and the condition usually appears between the second and…

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