Ectodermal dysplasia

The ectodermal dysplasias (EDs) comprise a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from embryonic ectoderm: the skin, hair, nails, eccrine glands, and teeth. Parents often complain of fever not subsiding by antipyretics and not sweating. Clinical examination reveals sparse, fair, brittle hair with alopecia, and sparse or absent eyebrows and eyelashes. They often have nail dystrophy and dental abnormality. Treatment consist of encouraging frequent consumption of cool liquids to maintain adequate hydration and thermoregulation and early dental evaluation and intervention and encouraging routine dental hygiene.