source: British Medical Journal
The Ehlers-Danlos syndrome is an inherited disorder of the connective tissue. It belongs to the same group of disorders as the Marfan syndrome, osteogenesis imperfecta, and pseudooxanthoma elasticum (McKusick, 1960). The main clinical features are: (1) fragility of the skin and of the dermal bloodvessels, (2) characteristic ” papyraceous ” scars, (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).
In this article evidence is presented that most babies with Ehlers-Danlos syndrome are born prematurely after premature rupture of foetal membranes. In all patients the fragility of foetal membranes (as shown by the degree of prematurity) is well correlated with the fragility of skin (as shown by the amount of skin-scarring). Finally, it is argued that in countries with good antenatal and premature-infant care an increase in the number of surviving Ehlers-Danlos patients is likely.
These observations were made on a group of 39 affected individuals-the largest series of Ehlers-Danlos patients so far recorded. The syndrome is generally regarded as emely rare, but this is due partly to a failure to recognize less obvious cases.
Postgraduate Medical School of London; Hammersmith Hospital
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