A Rapp-Hodgkin like syndrome in three sibs: Clinical, dental and dermatoglyphic study

Rapp-Hodgkin ectodermal dysplasia is an autosomal dominant disorder charact erized by distinctive craniofacies, cleft lip or palate, oligodontia or ano dontia, hypoplasia of the nails, and a decrease in or absence of the sweat glands and hair follicles. We have identified a family in which three child ren display clinical features similar to Rapp-Hodgkin syndrome. The father and two other sisters of the patient had normal facial features, but had sh ort stature and had dental anomalies, the latter suggestive of ectodermal d ysplasia. The overall clinical, dental, and dermatoglyphic findings of thes e patients are discussed in relation to reports of families with Rapp-Hodgk in syndrome. Clin Dysmorphol 8: 101-110 (C) 1999 Lippincott Williams & Wilk ins.