Hypotrichosis is a relatively common feature of a number of complex he
reditary syndromes. However, the isolated variant, called hereditary h
ypotrichosis simplex (HHS), is especially uncommon. We present a Spani
sh family with 8 of 19 persons covering 4 generations affected by HHS.
No associated ectodermal or other defects were noted. The pedigree wa
s compatible with an autosomal dominant inheritance with variable pene
trance.