Isolated congenital atrichia in an Omani kindred.

Congenital atrichia may occur isolated or with associated defects. The isolated variant is especially uncommon. Sporadic and familial cases have been rarely reported. We present a large inbred kindred from Oman where 22 affected members with this disorder were found over six generations. The male-to-female ratio was 1:1.4. No associated ectodermal or other defects were noted. A typical autosomal recessive mode of inheritance was documented. Though inbreeding was high, anticipated quasidominance was observed only once.