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#