Letterer-Siwe's Disease (Acute Disseminated Histiocytosis X): A Case Complicated by Disseminated Intravascular Coagulation and Responding to Heparin Therapy

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Abstract
Hemorrhagic skin lesions and thrombocytopenia are common manifestations of Letterer-Siwe disease.1-9 Both have been considered to be indicative of a grave prognosis.7,8 The case reported here satisfies the clinical criteria of Abt and Denenholz1 and the pathological criteria of Oberman.5 She presented with a hemorrhagic skin rash, thrombocytopenia, and a clotting profile indicative of intravascular coagulation. These abnormalities appeared to respond quickly to heparin and she is currently surviving some 8 months later, further improvement having been achieved by the administration of prednisone and cyclophosphamide. Disseminated intravascular coagulation is now known to be a feature of many diseases, but it has not previously been described in this disorder and may well contribute to the poor prognosis which has been associated with the onset of hemorrhagic manifestations.

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