Familial benign chronic pemphigus

Definition:

Familial benign chronic pemphigus, also known as Hailey-Hailey disease, is a rare, autosomal dominant skin disorder. The clinical features are uncomfortable skin blisters and vegetative lesions caused by friction. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history is detected. Mutations in ATP2C1 that encodes a secretory pathway Ca2+/Mn2+-ATPase in the Golgi apparatus impair desmosomal keratinocyte adhesion in Hailey-Hailey disease.

References:

[1]. Burhan Engin, et al. Hailey-Hailey disease: A fold (intertriginous) dermatosis. Clin Dermatol. 2015 Jul-Aug;33(4):452-5. [Content Brief]

[2]. J Dhitavat, et al. Calcium pumps and keratinocytes: lessons from Darier's disease and Hailey-Hailey disease. Br J Dermatol. 2004 May;150(5):821-8. [Content Brief]

[3]. Z Hu, et al. Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease. Nat Genet. 2000 Jan;24(1):61-5. [Content Brief]

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