Abstract: 【Abstract】 Objective To analyze clinical and pathological features of 6 cases of papular acantholytic dyskeratosis of the anogenital area. Methods Clinical and histopathological data were collected from 6 patients with papular acantholytic dyskeratosis of the anogenital area in the Department of Dermatology, Peking University Third Hospital from January 2013 to December 2022, and retrospectively analyzed. Results Among the 6 patients, 3 were males and 3 were females. Their age at onset ranged from 22 to 58 years. The time from the onset to diagnosis ranged from 1 to 18 months. All patients had no family history of similar skin lesions. The 6 patients all clinically presented with multiple clustered millet-sized flesh-colored or gray-whitish papules in the perianal and genital areas, with erosions and crusts on the surface of some lesions, sometimes accompanied by pruritus. Histopathological findings of skin lesions were similar, including hyperkeratosis with focal parakeratosis, as well as acantholytic and dyskeratotic cells scattered in the epidermis. The 6 patients were treated with topical glucocorticoids or tacrolimus ointment, etc., which exhibited varying efficacy, and some skin lesions persisted with repeated attacks. Conclusions Papular acantholytic dyskeratosis was a rare skin disease, and mostly occurred in the anogenital area. Its diagnosis should rely on both clinical manifestations and histopathological findings.

Key words: Acantholysis, Papular acantholytic dyskeratosis, Clinical manifestations, Histopathological features