Atrichia With Papular Lesions

    May 1986 in “ Archives of Dermatology
    Matthew Kanzler
    TLDR The condition is likely inherited in an autosomal-dominant pattern.
    The document reported a case of atrichia with papular lesions in an 11-year-old boy with common variable immunodeficiency, with similar findings in his father, suggesting an autosomal-dominant inheritance pattern. This rare form of ectodermal dysplasia involved the shedding of normal fetal hair within the first three months, sparing eyelashes, and the development of keratin-filled follicular cysts on the skin, particularly on the face, neck, scalp, and extremities, between ages 2 and 26. Teeth and nails remained normal, and the inheritance pattern was previously thought to be autosomal recessive.
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