Animal Model of Human Disease: Ehlers-Danlos Syndrome | oneedsvoice

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Animal Model of Human Disease: Ehlers-Danlos Syndrome

key information

source: The American Journal of Pathology

year: 1975

authors: Hegreberg GA

summary/abstract:

The Ehlers-Danlos syndrome (ED-S) of dogs and mink is an inherited disorder involving decreased tensile strength, laxity and hyperextensibility of the skin. The major dermal clinical manifestations are explained on the basis of a primary connective tissuLe defect, especially involving the collagenous component.

Fragility of the skin is the most characteristic and unique clinical trait of the ED-S in dogs and mink. The tensile strength of affected dog’s skin is reduced to 4% that of nonaffected dog’s skin, and the tensile strength of affected mink skin is reduced to approximately 8% that of nonaffected mink skin. Numerous lacerations of the skin have been recorded in affected dogs, and their skin often contains numerous broad thin scars. Skin lacerations heal adequately by primary intention healing, provided the free edges of the wound are approximated and retaining sutures placed deeply in the surrounding skin. The ED-S of both dogs and mink is genetically transmitted as an autosomal dominant trait.4 Penetrance of this dominant gene appears to be complete.

organisation: Washington State University

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