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Peculiar papular eruption on the vulva and inguinal folds
  1. Eman A El-Nabarawy,
  2. Nadia Saleh,
  3. Mona R E Abdel-Halim
  1. Department of Dermatology, Cairo University, Cairo, Egypt
  1. Correspondence to Dr Mona R E Abdel-Halim, Department of Dermatology, Cairo University, Cairo 11562, Egypt; abdelhalimmona{at}gmail.com

Abstract

Clinical question A 26-year-old woman presented with asymptomatic skin coloured to whitish keratotic papules on the vulva, inguinal folds and inner thighs of 6 years duration. She had no similar lesions elsewhere and no family history. A biopsy was taken from one of the papules.

Review the high-quality, interactive digital Aperio slide at http://virtualacp.com/JCPCases/jclinpath-2018-205434/ and consider your diagnosis.

What is your diagnosis?

  1. Darrier’s disease

  2. Hailey-Hailey disease

  3. Genital warts

  4. Papular acantholytic dyskeratosis of genitocrural area

  5. Pemphigus vegetans

What is your diagnosis?The correct answer is after the discussion.

Discussion Papular acantholytic dyskeratosis (PAD) of the genitocrural area is a peculiar acantholytic dermatosis with dyskeratosis localised to the vulva and inguinal folds with possible extension to the thighs and perineum.1 It mainly involves young or middle-aged females.1 Cases affecting males and involving genitalia, thighs and perianal region have been reported.2 It represents an allelic variant of Hailey-Hailey disease as heterozygous mutations in ATP2C1 gene (intron 5 and exon 24) have been reported in some cases.3

Clinically, PAD of the genitocrural area is typically characterised by solitary or grouped keratotic flesh coloured or white papules.1 Other clinical presentations include: vesicles, bullae, patches and plaques.4 Although the lesions are mainly asymptomatic, patients may experience variable degrees of itching or burning sensation.

Histopathologically, the lesions show hyperkeratosis, hypergranulosis, acanthosis and acantholytic dyskeratosis in the spinous layer (figure 1). Typically, direct immunoflourescence (DIF) is negative.5

Figure 1

Photomicrograph representing histopathological findings: hyperkeratosis, hypergranulosis, acanthosis and acanthloytic dyskeratosis involving the spinous layer; 254×143 mm.

The most important differential diagnoses of this sporadic condition include: Darier’s and Hailey-Hailey, both of which are familial. Hailey-Hailey involves also other flexural areas and presents with eroded crusted lesions with foul odour. Pathologically, the acantholytic process in PAD of the genitocrural area is rather focal compared with the broad zone of acantholysis in Hailey-Hailey, and columns of parakeratosis overlying suprabasilar acantholysis are characteristic of Darier’s disease. Although, some cases of PAD of the genitocrural area can present with vesicles and bullae, the presence of dyskeratotic cells and the negative DIF speaks against pemphigus.

Treatment options for this condition include topical and systemic retinoids. Cryotherapy and laser have been also reported to be effective. Topical steroids might be effective in reducing pruritus.

  • dermatopathology
  • diagnosis
  • genitourinary pathology

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Footnotes

  • Handling editor Iskander Chaudhry.

  • Contributors All authors have contributed to the authorship.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

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