Histologic examination showed suprabasal vesicle formation with extensive acantholysis characterized by numerous detached keratinocytes within the blister cavity. The epidermis displayed downward growth, with corresponding formation of dermal villi. The papillary dermis showed a minimal infiltrate of mixed inflammatory cells.
Benign familial chronic pemphigus is a rare autosomal dominantly inherited dermatosis originally described by Hailey and Hailey1 in 1939. About 70% of patients report a family history of the genodermatosis.2 Although the genetic defect is not known, the chromosomal defect has been localized to chromosome 3q, a site nonallelic to the histologically similar genodermatosis of Darier disease.3,4 Further information from our patient revealed a family history of Hailey-Hailey disease: her sister, father, father's sister, and paternal grandfather were also affected, each with varying severity. Her son, aged 26 years, is unaffected.
Vulvar Verrucous Papules. Arch Dermatol. 1999;135(2):203–b–208. doi:10-1001/pubs.Arch Dermatol.-ISSN-0003-987x-135-2-dof0299
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