Ectodermal dysplasia associated with sickle cell disease.

Case Rep Dent

Department of Post-Graduation, Master Program in Integrated Dentistry Science, University of Cuiabá, Avenida Manoel José de Arruda No. 3.100, 78050-000 Cuiabá, MT, Brazil.

Published: October 2014

Ectodermal dysplasia and sickle cell anaemia are inherited disorders that affect, respectively, the tissues derived from the embryonic ectoderm and the production of erythrocytes by the bone marrow. The simultaneous occurrence of both disorders is extremely rare. This is a case of both ectodermal dysplasia and sickle cell anaemia reported in a 6-year-old. The patient had been diagnosed with sickle cell anaemia for only six months when he sought treatment presenting with the following: hypotrichosis, dry skin, periocular hyperpigmentation, protruding lips, hypodontia, and morphologically altered teeth. The clinical features combined with his medical history led to the diagnosis of ectodermal dysplasia. Dentists should be prepared to recognise patterns that escape normality to aid in the diagnosis of systemic changes, even in patients with other previous diagnoses.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4197856PMC
http://dx.doi.org/10.1155/2014/314391DOI Listing

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