Aneurysms and hypermobility in a 45-year-old woman.
EDS type IV presents a diagnostic and therapeutic challenge to the primary care physician, surgeon, and rheumatologist. In patients for whom the diagnosis is known, avoidance of trauma, contact sports, or strenuous activities, joint bracing and protection, and counseling on contraception are helpful preventive strategies. In patients presenting with vascular, gastrointestinal, or obstetric complications, a history of hypermobility and skin fragility (easy bruising, abnormal scarring, poor wound healing) should lead to a suspicion of this diagnosis, and to caution in the use of certain invasive diagnostic and operative techniques. Efforts should be made to examine family members. Most importantly, when caring for such patients, the acute onset of headaches, chest pain, shortness of breath, and abdominal pain should arouse suspicion of a potentially catastrophic vascular or visceral event.
Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Ohio 44195, USA.
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Santos-Ocampo
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Hoffman
D000783:Aneurysm / Q000209:etiology*; D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D005260:Female; D006499:Hepatic Artery; D006801:Humans; D007593:Joint Instability / Q000209:etiology*; D008382:Marfan Syndrome / Q000175:diagnosis; D008875:Middle Aged; D014657:Vasculitis / Q000175:diagnosis