Ektodermal displazi

Abstract

Ectodermal dysplasia (ED) is a rare, hereditary, congenital disease that affects teeth, skin, hair, nails, eccrine and sebaceous glands. Symptoms of ED are hypohidrosis or anhidrosis, heat intolerance, dental and nail abnormalities, hypotrichosis, inability of lacrimation, thinning and hypopigmentation of skin. ED has got three types: Hypohidrotic (unhydrotic), hidrotic (clouston type) and EEC syndrome (ectrodactyly, ED, cleft lip and palate). In hypohidrotic type there are decreased or absent sweating glands, abnormal dental structure and hypotrichosis.Our patient is 7.5 years old, male. When he come to our clinic, he has got afebrile seizure, inabilitiy to sweat, decreased tears and abnormal skin presentation. When we focus on history of patient's symptoms, we decided that this signs and symptoms are related to hypohidrotic ED and perinatal asphyxia sequel.In this case, we diagnosed ED with history and typical signs of patient. We present this case because of its rare and congenital disease.