title: Papular acantholytic dyskeratosis of the anogenital and genitocrural area: case series and review of the literature.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Maher
authors.initials: M
authors.lastname: Al-Muriesh
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Bilal
authors.initials: B
authors.lastname: Abdul-Fattah
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Mengjie
authors.initials: M
authors.lastname: Zhao
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Siyuan
authors.initials: S
authors.lastname: Chen
authors.affiliation: Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
authors.forename: Changzheng
authors.initials: C
authors.lastname: Huang
mesh_terms: D000051:Acantholysis / Q000473:pathology*; D000328:Adult; D001004:Anus Diseases / Q000473:pathology*; D002648:Child; D005260:Female; D052776:Female Urogenital Diseases / Q000473:pathology*; D006801:Humans; D008297:Male; D052801:Male Urogenital Diseases / Q000473:pathology*; D008875:Middle Aged; D055815:Young Adult
keywords: Darier disease; Hailey-Hailey disease; anogenital; genitocrural; papular acantholytic dyskeratosis