Abstract

Papular acantholytic dyskeratosis (PAD) of the genitocrural area is a rare but distinct histopathologic cutaneous disorder of the genitalia and can present with a variety of clinical manifestations. We describe a case of PAD of the genitocrural area in a 60-year-old, postmenopausal female with a Darier-predominant histological pattern. The patient initially presented with increased vulvar/vaginal dryness and pruritus and was treated with topical zinc oxide and topical vaginal estrogen. One month later, she again presented with severe vulvar pain, multiple deep vulvar ulcerations with rolled edges, and a 5mm raised, verrucous, round lesion on the lower left labia majora. She was treated with topical lidocaine and valacyclovir with minimal resolution. A punch biopsy of the lesion was obtained. Histologic examination showed acanthosis, papillomatosis, and marked dyskeratosis (including scattered corps ronds and grains) with only focal acantholysis. Immunohistochemical stains for HSV-1 and HSV-2 were negative. There was no personal or family history of Hailey-Hailey or Darier disease. To our knowledge, there are limited case reports describing PAD of the genitocrural area with severe pain and a Darier-predominant histological pattern. Though the exact pathophysiology of PAD of the genitocrural area is unknown, a recent case study described somatic mosaicism of ATP2A2 mutations (commonly associated with Darier disease) as a potential genetic cause for PAD. Dermatopathologists should be aware of this entity because it is important to distinguish PAD of the genitocrural area from cases of segmental Darier and Hailey-Hailey disease. Careful assessment of clinical presentation, disease course, and family history play an important role in conjunction with characteristic histopathological findings to make a more definitive diagnosis of PAD in the genitocrural area.

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