Papular acantholytic dyskeratosis of the anogenital and genitocrural area: case series and review of the literature.

BACKGROUND: Papular acantholytic dyskeratosis (PAD) of the anogenital/genitocrural area is described as a rare distinct clinicopathological entity known to dermatopathologists, although its characteristic histopathologic pattern resembles both Hailey-Hailey disease and Darier disease. The objective of this study is to describe the clinical characteristics, histopathologic features and response to treatment of PAD.

METHODS: We report in detail six cases of PAD. A literature search of the keyword 'papular acantholytic dyskeratosis' was performed on Google scholar and PubMed, 21 cases of this entity were found. A total of 27 patients including our six cases are reviewed in this study.

RESULTS: The mean age at diagnosis was 38.8 years with a male to female ratio of 0.8 : 1. Clinically, papular lesions (55.6%) are the typical manifestation of PAD, and the anogenital area (63%) is the most commonly involved site. Lesions were resistant to topical steroids, subcutaneous interferon and antibiotics while one case showed complete resolution of the lesions after retinoid therapy. Laser therapy showed good results in one case. None of the patients had spontaneous remission.

CONCLUSION: Awareness of the clinicopathological hallmarks herein may be important to avoid underdiagnosis of PAD and may contribute to understanding the pathogenesis of this rare disease.