Classical Ehlers-Danlos Syndrome
摘要
Classical EDS (cEDS) is described with comments on the 10 less common subtypes of EDS. cEDS is the amalgamation of hEDS and vEDS: There are cEDS type I and type II, differentiated by severity. Type I had more severe joint hypermobility and skin laxity. The criteria used to distinguish type II from type I was the absence of widened atrophic scars in cEDS type II. cEDS is estimated to occur in approximately 1/20,000 people, but it is often unrecognized. With cEDS, the major diagnostic criteria include skin hyperextensibility, widened atrophic scars, and joint hypermobility. The skin is the key in establishing the diagnosis of cEDS. It is reliable and reproducible. The skin is more hyperextensible than is usually seen in other hEDS/HSD patients. Skin is very soft (“velvety” or “doughy” similar to patients with massive weight loss) and fragile, which leads to atrophic scarring. The skin snaps back easily upon release. Wounds split open with little trauma, or even spontaneously, with little bleeding and scars that widen over time to create characteristic atrophic or “cigarette paper” scars. Easy bruising and poor wound healing are characteristic.