Atrichia and Papular Lesions: Report of a Case

    January 1992 in “ Dermatology
    Cosimo Misciali, Antonellá Tosti, Pier ­Alessandro Fanti, P. Borrello, Bianca Maria Piraccini
    TLDR A 4-year-old girl has a rare condition causing hairlessness and skin bumps, but normal teeth and sweating.
    A case of atrichia with papular lesions was reported in a 4-year-old girl who had been completely hairless on the scalp since birth, except for some dark, shiny, coarse hair in the frontoparietal region. Sparse eyelashes and eyebrows were noted, and numerous papular lesions appeared on the scalp, cheeks, and neck by the second year of life. Despite these symptoms, her teeth and sweating function were normal, and there was no relevant family history. Histological examination revealed keratin-filled cysts and tubular epithelial structures without hair bulbs in the bald scalp, with sebaceous and outer root sheath differentiation. The superficial dermis contained horny cysts similar to those on the cheeks.
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