Papular Acantholytic Dyskeratosis of the Vulva

Abstract

We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years follow-up, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.

Papular acantholytic dyskeratosis of the vulva

“…It is usually asymptomatic but sometimes pruritus may appear. Most of the reported cases were women and occurred on the vulva and in the perianal area, but there was a report of male patient with the lesion on his penis 5–9 . Clinically, this should be differentiated from vulvar papules of infectious origin, Hailey–Hailey disease, Darier's disease localized to the genital area, and premalignant and malignant lesions.…”

“…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.…”

Effective treatment of papular acantholytic dyskeratosis with oral isotretinoin

“…In 1972 Ackerman introduced the term focal acantholytic dyskeratosis 5 to describe a peculiar histological pattern, characterized by suprabasal cleft, hyperkeratosis, parakeratosis, acantholytic and dyskeratotic cells, including corps ronds . This pattern is classical of DD, but is also found in other genetic diseases such as benign familial pemphigus, and in acquired dermatosis such as Groover (also named transient acantholytic dermatosis), 2 warty dyskeratoma and PAD. The latter was first described in the genital region.…”

“…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.…”

Peculiar papular eruption on the vulva and inguinal folds

“…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.…”