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Congenital Atrichia with Papular Lesions
Congenital atrichia with papular lesions is a rare autosomal recessive disorder in which there are multiple, widely distributed milia-like papules in addition to a complete absence of hair that is noted within a few months of birth. Patients have hair at birth but once it falls out it is not replaced. The alopecia is non-cicatricial but non-reversible and is due to mutations in the hairless gene (HR), which encodes a transcriptional corepressor of multiple nuclear receptors and Notched target genes. Histologically, there is replacement of the middle and lower portions of the hair follicle by keratinizing cysts.