164 A family case of alopecia universalis congenita is not linked to hairless gene

Y. Takahashi, O. Mori, M. Morita, and T. Hashimoto Dept. of Dermatology, Kurume University School of Medicine, Fukuoka, Japan

Inheritied forms of hair loss without associated skin or internal abnormalities are rare diseases. Two autosomal recessive types of this condition have been described: alopecia universalis congenita and atrichia with papular lesions. The two diseases have been shown to result from a mutation in the human hairless gene. We report a Japanese family of alopecia universalis congenita with autosomal dominant mode of inheritance, in which a 28-year-old father and his two sons were affected. No associated ectodermal defects were noted. In mutation analysis for the father, no mutation was identified in hairless gene. These results suggest that there may be another gene responsible for alopecia universalis congenita.