Ehlers-Danlos Syndrome Type IV: A Genetic Disorder in Many Guises

Byers, Peter H.
September 1995
Journal of Investigative Dermatology;Sep95, Vol. 105 Issue 3, p311
Academic Journal
Ehlers-Danlos Syndrome (EDS) type IV reminds of a disorder whose dermatologic findings are more curiosity than concern but whose systemic complications can be and generally are lethal. EDS type IV is almost certainly the acrogeria and is known as the vascular-ecchyrnotic form of EDS. The dermatologic manifestations of EDS type IV are variable. In some people, acrogeria is striking and the aged appearance of the skin, particularly of the hands and feet, generates comment although not always medical referral. EDS type IV is only one of several varieties of EDS. Because the molecular basis of these disorders is different and the clinical complications of each are distinct, it is important that clinical diagnosis is established by appropriate studies.


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