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A patient with cutaneous lesions consistent histologically, ultrastructurally, and immunohistochemically with angioendotheliomatosis is described. The cutaneous lesions appeared 14 months prior to the diagnosis of histiocytic lymphoma of the retroperitoneum. Both the cutaneous lesions and the lymphoma responded well to systemic chemotherapy consisting of cyclophosphamide, hydroxydoxorubicin, vincristine, and prednisone.
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