Hailey-Hailey disease
benign chronic pemphigus
Recurrent eruption of vesicles and bullae involving predominantly the neck, groin, and axillary regions is characteristic of this disorder.
Microscopical synopsis
numerous acantholytic cells
suprabasal type of blister formation strikingly resembling that in pemphigus vulgaris malignus.
Etiology
germline mutations in the ATP2C1 gene (MIM.604384) causing a defect in keratinocyte cohesion
References
Foggia L, Hovnanian A. Calcium pump disorders of the skin. Am J Med Genet. 2004 Nov 15;131C(1):20-31. PMID: 15468148
Dhitavat J, Fairclough RJ, Hovnanian A, Burge SM. Calcium pumps and keratinocytes: lessons from Darier?s disease and Hailey-Hailey disease. Br J Dermatol. 2004 May;150(5):821-8. PMID: 15149492