Atrichia With Papular Lesions

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.