HEIHACHIRO KASHIWAGI, M.D.; JEANNE M. RIDDLE, PH.D.; JOSEPH P. ABRAHAM, M.D.; BOY FRAME, M.D., F.A.C.P.
Ehlers-Danlos syndrome is a heritable disorder of connective tissue characterized by hyperelastic, fragile skin and hyperextensible joints (1). The basic defect is thought to be a disturbance of the "wicker-work" of collagen fibers (2).
Included among many other features of this condition are easy bruising and hemorrhagic tendency. Indeed, frequent hematoma formation was one of the salient features of the case originally described by Ehlers in 1901 (3). Bleeding may occur at any site and contribute to considerable morbidity. The literature describes cases of gastrointestinal bleeding (4, 5), postpartum bleeding (4), and hematomas simulating hemophilia (3, 6, 7).
KASHIWAGI H, RIDDLE JM, ABRAHAM JP, FRAME B. Functional and Ultrastructural Abnormalities of Platelets in Ehlers-Danlos Syndrome. Ann Intern Med. 1965;63:249–254. doi: 10.7326/0003-4819-63-2-249
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Published: Ann Intern Med. 1965;63(2):249-254.
Hematology/Oncology, Hospital Medicine, Platelet Disorders.
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