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December 2003

Infants With "Hair Today That's Gone Tomorrow"An Inherited Atrichia?

Arch Dermatol. 2003;139(12):1644-1645. doi:10.1001/archderm.139.12.1644

OUR UNDERSTANDING of the underlying molecular changes that lead to genetic hair loss in infants has significantly increased in the last several years. Three forms of inherited atrichia, or total absence of hair, have been shown to result from mutations in 3 different genes. Atrichia in association with severe T-cell immunodeficiency and nail dystrophy has been described in 2 sisters.1 This form of atrichia, the human homologue of the nude mouse, results from deletion of the human WHN gene. The other 2 forms of inherited atrichia, atrichia with papular lesions (APL), which is the human homologue of the hairless mouse (Online Mendelian Inheritance in Man [OMIM] 209500),2 and vitamin D–dependent rickets with atrichia (VDDR IIA; OMIM 277440),3 result from mutations in the hairless and vitamin D receptor genes, respectively. However, as demonstrated by Zlotogorski et al4 in this issue of the ARCHIVES, the cutaneous phenotypes of these 2 genetically diverse conditions are identical.

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