Benign Persistent Papular Acantholytic and Dyskeratotic Eruption of the Vulva: A Case Report

Abstract

We report a case of 44-year-old woman with persistent pruritic papules on the left and right labium majus of the vulva. Histopathologic examination of the vulvar biopsy specimen revealed a suprabasal separation of the epidermis with acantholysis and dyskeratosis.

Papular acantholytic dyskeratosis of the vulva

“…It is also found incidentally in a variety of skin lesions including condyloma acuminatum, squamous cell carcinoma, malignant melanoma and pityriasis rosea 4 . Papular acantholytic dyskeratosis (PAD) is a rare skin disease, characterized by skin‐to‐white colored keratotic papules in the genital area 5–7 . We report an uncommon case of papular acantholytic dermatosis of the vulva in a 63‐year‐old woman.…”

Papular acantholytic dyskeratosis of the anogenital area with positive direct immunofluorescence results

“…That patient had the characteristic histopathological changes, including acantholyic and dyskeratotic cells in the hyperkeratotic and parakeratotic epidermis, which are noted in Hailey-Hailey disease. 4 DIF microscopy was performed in most cases of acantholytic dyskeratotic dermatosis and was found to be negative. The authors regarded it as a new entity and named this disease 'papular acantholytic dyskeratosis of the vulva'.…”

“…4 In addition, immunoglobulins and C3 have been found in the epidermal intercellular areas of some patients with Darier's disease by DIF. Both Hailey-Hailey disease and Darier's disease confined to the vulvocrural area have been described.…”

“…4 In addition, immunoglobulins and C3 have been found in the epidermal intercellular areas of some patients with Darier's disease by DIF. 1,3,4 Grover's disease also shows similar histopathological features, and some patients with Grover's disease have been reported to show positive DIF findings. 4,5 Figure 1 The numerous smooth whitish papules that coalesced into plaques in some areas on the labia majora and perineum.…”

Effective treatment of papular acantholytic dyskeratosis with oral isotretinoin

“…Papular acantholytic dyskeratosis (PAD) is a rare skin disease, characterized by pruritic keratotic papules, mainly in the genital skin 1–3 . Histologically, typical features of Darier's disease (DD) are found, but PAD is an acquired condition with localized lesions, when compared to DD.…”