Ehlers-Danlos syndromemedical dictionary

<dermatology, rheumatology> A connective-tissue disorder characterised by hyperelasticity of skin, poor wound healing, hyperextensibility of joints, soft-tissue calcifications (spheroids), vascular lesions: aortic dissection, aneurysms, rarely: tortuous arch, ectatic pulmonary artery, tissue fragility most likely to be haematomas, aortic rupture after angiography, clinical types: gravis, mitis, benign hypermobile, ecchymotic, X-linked, associated with medullary sponge kidney

Inheritance: autosomal dominant, X-linked.

Synonyms: dermatosparaxis.

(12 Dec 1998)

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