A 12-year-old Boy With Multiple Bruises and a Dislocated Knee But No Reported Injury

Baruch, Marlene B; Beck-Little, Rebecca

doi:10.1016/s0099-1767(03)00293-9

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2008

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“…It comprises a heterogeneous group of diseases characterized by laxity and abnormal extensibility of skin and joints; the degree of severity is the criterion used to distinguish different EDS subtypes [18]. Joint hypermobility can be severe and sometimes patients have a history of recurrent joint luxations [19,20]. The skin is soft and velvety and can be stretched for some centimeters; when relaxed, it returns to the original site.…”

Section: Discussionmentioning

confidence: 99%

HEMARTHROSIS DUE TO A RARE CAUSE OF HEMORRHAGIC DIATHESIS: Ehlers-Danlos Syndrome

Giordano

Vecchio

Scaraggi

et al. 2008

Pediatric Hematology and Oncology

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The authors report a case of hemarthrosis complicated by severe anemia related to a congenital connective tissue disease: Ehlers-Danlos syndrome. A boy fell down and suffered tumefaction of both knees with bilateral rupture of the rotula tendon. He underwent surgical reinsertion of each tendon on the rotula. He later showed an unexpected ongoing hematic effusion, with severe anemia. He was screened for coagulation disorders with no results. On taking a more detailed history and investigating the patient's phenotypical features, the authors diagnosed Ehlers-Danlos syndrome, hypermobile variant. The hemarthrosis and anemia were thus concluded to be consequences of excessive tissue fragility due to a congenital connective tissue disease.

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Section: Discussionmentioning

confidence: 99%