A 36-year-old woman had a 6-year history of recurrent panniculitis with development of an angiocentric and angiodestructive cutaneous T-cell lymphoma (CTCL) of the helper cell phenotype. She subsequently developed a rapidly progressive fatal syndrome characterized by cytophagocytic histiocytosis and hyperlipidaemia. Cytophagocytic histiocytosis has previously been reported in association with panniculitis, malignancy and infection, but not with CTCL and the precise relationship between panniculitis, CTCL, cytophagocytic histiocytosis and hyperlipidemia is unclear.
A case is reported, believed to be the first, of a patient with a long‐standing cutaneous T‐cell lymphoma, in whom disseminated plasma cell myeloma subsequently developed shortly before the patient died. It is postulated that, rather than to a chance association, the second B‐cell neoplasm evolved under the sustained inducer‐cell activity of the malignant lymphocytes of the cutaneous T‐cell lymphoma.
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