Ehlers-Danlos type IV is characterized by joint hyperextensibility confined to the upper extremities, minimal skin hyperelasticity, and severe bleeding diatheses. Unlike the other types of Ehlers-Danlos syndrome, in type IV or the ecchymotic variety, the connective-tissue defect includes blood vessels, most often major arteries. A young man with this disorder was recently seen.
Report of a Case
A healthy 19-year-old man had large ecchymoses on his right arm while exercising on parallel bars. Because of the discomfort, he took 325 mg of aspirin every six hours. Two days later, while doing sit-ups, he noted acute, excruciating abdominal wall pain in the right hypogastrium. During the next 24 hours, a 10-cm ecchymotic area in the epigastrium developed, with a protuberant rectus sheath. The patient was referred to our hematology branch for examination the same day.His history disclosed easy bruising since childhood. At age 6 years, he had a spontaneous rupture