ECTODERMAL ABNORMALITIES IN KABUKI-SYNDROME

We describe a girl with Niikawa-Kuroki (Kabuki) syndrome (NKS) with co nical incisors, hypodontia, hypoplastic nails, and brittle hair. Abnor mal teeth are common in NKS and support a hypothesis of autosomal domi nant inheritance of the syndrome [Halal et al., 1989; Silengo et al., 1996]. Hair abnormalities have never been investigated in NKS. The ect odermal involvement in NKS could represent an important clue for the u nderstanding of the pathogenesis of this syndrome. (C) 1997 Wiley-Liss , Inc.