Syndrome of Ectrodactylism, ectodermic dysplasia and lip-cleft palate

Revista Cubana de Estomatología

Sepúlveda Infante, Rogelio¹  Torre Martinez, Hilda H. H.¹  Salazar Fernández, Mario A.¹  Menchaca Flores, Pedro N.¹  Romero de León, Elías¹ 

[1] Facultad de Odontología, Monterrey, México

关键词: síndrome;    ectrodactilia;    displasia ectodérmica;    labio-paladar hendidos;    síndrome EEC;   

DOI  :  

学科分类：基础医学

来源: SciELO

【 摘 要 】

In present paper is reported the case of a patient presenting with the complete triad of Ectrodactylism syndrome, ectodermic dysplasia and lip-cleft palate, to mention the clinical findings present in assessments as well as the treatments applied. The syndrome is characterized by the triad Ectrodactylism, ectodermic dysplasia and cleft (EEC) and it is autosomal dominant with incomplete penetration and a variable expressiveness. This group of signs and symptoms may be cofounded with other clinical entities, leading to a mistake diagnosis of patient. Lip cleft and lack of lacrimal duct is not a usual combination in other conditions. This is the clinical case of a male patient aged 12 with the syndrome of: Ectrodactylism, ectodermic dysplasia and lip-cleft palate. Clinical manifestations reported in literature are mentioned emphasizing the maxillofacial and dental manifestations. The two brothers of patient were normal or free of this syndrome. It is very important that the professionals charged of oral and maxillofacial health, as well the Stomatology students of Bachelor degree of postgraduate level, be able to identify, diagnose and to work in a multidisciplinary way to arrive to definite diagnosis of patients presenting with EEC syndrome and to apply the appropriate treatments.

【 授权许可】

CC BY-NC