Abstract

Abstract—EEC syndrome is an ectodermal dysplasia with primary involvement of the ectodermal oral and epidermal appendages. It is an autosomal, dominantly inherited condition which has three cardinal signs: ectrodactyly, ectodermal dysplasia, and cleft palate and/or harelip. Skin, hair, teeth, and nails can be affected. In addition to the cardinal symptoms, anomalies in the lacrimal ducts or urogenital anomalies can be part of the syndrome. The differential diagnosis includes other ectodermal dysplasias with cleft palate and with or without harelip such as the Rapp-Hodgkin syndrome and the AEC (ankyloblepharon, ectodermal dysplasia, and cleft palate with or without harelip) syndrome.

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