Ectrodactyly, ectodermal dysplasia, and cleft lip or palate syndrome: A review with rare case report

Abstract

Ectrodactyly, Ectodermal dysplasia, and cleft lip or palate (EEC) syndrome is a rare ectodermal dysplasia syndrome, and very few cases are reported in the Indian literature. Ectodermal dysplasia constitutes a heterogeneous group of disorder characterized by various defects in hair, nails, teeth, and sweat glands. The term “EEC syndrome” was coined by Rudiger et al. in 1970. The syndrome results from developmental anomalies that involve both ectodermal and mesodermal tissues and consists of EEC. It is a complex multiple congenital anomalies syndrome in which any of the three cardinal signs are present in variable expression, specific clinical pattern of defects may lead to correct diagnosis. Prosthodontic rehabilitation may aid in restoring the normal function and esthetic in such patients. The present article reviews the literature on EEC syndrome and reports on the Indian kindred consisting of two siblings with ectodermal dysplasia, one of them (a boy) presented with EEC syndrome.