source: Archives of Disease in Childhood
King-Lewis FL, Polunin IV
The Ehlers-Danlos syndrome is a congenital abnormality of the skin, of which the following may be considered the chief characteristics: (1) Abnormal friability of the skin and subcutaneous tissue; (2) loose and hyperelastic skin; (3) hyperextensibility of joints; (4) the presence of nodules of various types in the skin ; (5) well-marked epicanthic folds.
The clinical picture is not invariably complete and, as in other congenital conditions, the incidence of associated congenital abnormalities is higher than in normal subjects. The friability of the skin is nearly always evident from the history of these patients. They are described as being subject to cuts or even large gaping wounds from the most trivial injuries, and a glance at the skin of their knees or foreheads will show scarring of an unusual degree. The laxity of the skin is easily demonstrable as a rule by the ease with which a fold may be drawn away from the deeper tissues, and its lack of attachment is suggested by the absence of skin. creases which, according to Le Gros Clark (1945) are due to the attachment of the skin to deep fascia by strands of collagen fibres.
King Edward Memorial Hospital
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