Familial Benign Pemphigus (Hailey-Hailey Disease) Workup

Updated: Jul 07, 2022
  • Author: Thomas N Helm, MD; Chief Editor: Dirk M Elston, MD  more...
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Laboratory Studies

Microscopic examination reveals intraepidermal and suprabasilar acantholysis, as shown in the image below. Elongated papillae (villi) extend into lacunae, and a single layer of basal cells lines the villi.

Acantholysis at all levels of the epidermis (hemat Acantholysis at all levels of the epidermis (hematoxylin and eosin stain, original magnification X20).

Many layers of detached keratinocytes (acantholysis) exist and appear similar to a dilapidated brick wall (clumped acantholytic cells have only a few intact intercellular bridges holding the keratinocytes together).

Unlike pemphigus vulgaris, direct immunofluorescence in familial benign pemphigus is negative. The Nikolsky sign is negative, and Tzanck preparation fails to reveal giant cells and syncytia formation characteristic of herpes virus infection.

Serologic studies for familial benign pemphigus fail to reveal circulating autoantibodies, unlike pemphigus vulgaris in which antibody titers correlate with disease activity.


Histologic Findings

Histologic findings in familial benign pemphigus are suprabasilar and widespread acantholysis.