Work

Unusual manifestations of ectodermal dysplasia-syndactyly syndrome type I in two Yemeni siblings

Year: 2015

Type: article

Abstract: Ectodermal dysplasias (EDs) are a group of genodermatoses characterized by malformations of tissues derived from the ectoderm, including the skin, its appendages (hair, nails, sweat glands), teeth, and the breasts. Ectodermal dysplasia syndactyly syndrome (EDSS) is a rare, newly described type of ED involving syndactyly. We report 2 Yemeni siblings with typical EDSS manifestations, including bilateral, partial cutaneous syndactyly of the fingers and toes; sparse, coarse, brittle scalp hair, eyebrows, and eyelashes; and conical, widely spaced teeth with enamel notches. In addition, the siblings presented with other features hitherto not described for this syndrome, such as adermatoglyphia, onychogryphosis, hypoplastic widely spaced nipples, hypoplastic thumbs, and red scalp hair.

Source: Dermatology Online Journal

DOI: 10.5070/d3211025435

Author: Alshami Mohammad

Institution: Sana'a University

Language: English

Topic: dental development and anomalies

Subfield: General Dentistry

Field: Dentistry

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $300