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KMID : 0378019700130020088
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New Medical Journal
1970 Volume.13 No. 2 p.88 ~ p.91
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Ehlers-Danlos Syndrome
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Abstract
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The Ehlers-Danlos syndrome is an inherited disease of the connective tissue. It belongs to the same group of disorders as the Marfan & syndrome, osteogenesis imperfecta and pseudoxanthoma elastiium (Mc Kusick, 1960).
The main clinical features are: (1) fragility of skin and of dermal blood vessels, (2) characteristic "papyraceus" scar, (3) hyperextensible skin, and (4) hypermobile joints.
The basic defect is believed to lie in the organization of collagen into a stable collagen `Wickerwork" (Jansen, 1955).
Because the initial symptoms and the manifestations of this condition vary, it is important for all physician to be aware of this entity and its multiple system involvement.
Recently, we have experienced two cases of Ehlers-Danlos syndrom. Both patients are female and their presenting symptoms and singns are hyperela & ticity and fragility of skin and hypermobility of joints associated with congenital dislocation of both hip joints in one case, and subluxation of both radiohumeral joints and congenital dislocation of left hip joint in other.
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KEYWORD
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