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Ehlers-danlos syndrome

Ehlers-danlos syndrome

(Science: 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.

Synonym: dermatosparaxis.


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