Hypohydrotic Ectodermal Dysplasia in Black Africans

Yıl 2013, Cilt: 10 Sayı: 3, - , 01.09.2013

Modupeola O Samaila Sunday O Ajike Olufemi G Ogunringe Turaki T Mohammed

Öz

Ectodermal dysplasia is an uncommon genodermatosis which may be associated with mental retardation. The diagnosis is often missed in newborns or delayed due to its varied overlapping clinical presentation in affected individuals. We report two maternal blood related males with complaints of heat intolerance since birth, lack of sweating and dry scaly skin. Both had normal developmental milestone and mental acuity. The treatment is symptomatic and patient dependant while overall management requires a multidisciplinary team approach.

Anahtar Kelimeler

Hypohidrotic, ectodermal dysplasia, hypodontia

Hypohydrotic Ectodermal Dysplasia in Black Africans

Öz

Ektodermal displazi mental retardasyon ile ilişkili nadir genodermatozisdir. Tanı sıklıkla yenidoğanlarda atlanılır ve kişileri etkiliyen diğer klinik durumlar nedeniyle geç konulur. Biz terleme eksikliği ve kuru deri nedeniyle doğumda ısı intoleransı gelişen 2 erkek hasta bildirdik. Tedavi semptomatik ve multidisipliner bir yaklaşım ile hastaya göre değişmektedir

Anahtar Kelimeler

Hipohidrotik, ektodermal displazi, hipodonti

Kaynakça

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