HAILEY-HAILEY DISEASE (FAMILIAL BENIGN PEMPHIGUS)
Hailey-Hailey disease is characterized by recurrent eruptions, usually in intertriginous areas, axillae, groin or on the neck. Vesicles, erosions, and crusts may be present in these erythematous lesions.

Suprabasal acantholysis is the characteristic pathologic finding. Severe acantholysis is seen in some lesions, and, in the proper clinical setting, this finding is diagnostic. The pathology is some biopsies may only consist of a suprabasal slit with minimal acantholysis, and such a biopsy may be indistinguishable from that seen in pemphigus vulgaris. Some cases of Grover's disease (transient or not-so-transient acantholytic dermatosis) may share pathologic features with Hailey-Hailey disease though dyskeratosis is seen more commonly in Grover's disease.


This 28 year old female has a long history of recurrent eruptions in the intertriginous areas. This is a biopsy from beneath a breast.



 
Composite low power view.

 
High power view from the right side of the composite low power picture. Severe acantholysis ('dilapidated brick wall' appearance) is diagnostic in this clinical setting.
High power view from the left side of the picture. The pathology seen in this field overlaps that which can be seen in pemphigus vulgaris. The clinical presentation, however, is distinctive.

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