[Neuroectodermal dysplasia in 2 Uzbek families].

The authors present the clinical and genealogical description of 6 patients with neuroectodermal dysplasia diagnosed by a medical expedition party in Khankin district of the Khorezm region. Unique combination of clinical signs (total alopecia, microcephalia, oligophrenia, hyperhydrosis and hypogenitalism) helped differentiating the syndrome from other well-known hereditary neuroectodermal dysplasias. The nature of the syndrome segregation in the families suggested its autosomal-recessive mode of inheritance.