Panniculitis associated with cutaneous T-cell lymphoma and cytophagocytic histiocytosis

Abstract

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.