[The Ehlers-Danlos syndrome type III and type IV]. 2007

M H Roqué, and C M Roqué
Consultorio de Reumatología, Cátedra de Historia de la Medicina, UNC, FCM.

The Ehlers-Danlos Syndrome (EDS) is a collagen disease characterized by joint hypermobility, skin hyper-extensibility and generalized tissue fragility. It is usually inherited in an autosomal dominant manner and they differ from each other in gene mutation involved in the structure of different types of collagen. We report two female patients with a diagnosis of EDS Type III and Type IV with different clinical manifestations, evolution and prognosis. The first case, EDS Type III, presented marked joint hypermobility and skin hyper-extensibility, and the second case, EDS Type IV, arterial rupture leading to extensive bruising, obstetrics complications and skin hyper-extensibility.

UI MeSH Term Description Entries
D004535 Ehlers-Danlos Syndrome A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability. Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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