Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis

Abstract

A 43-year-old Maori man presented with a 1 month history of malaise, weight loss, anorexia, arthralgia, recurrent fever and tender erythematous subcutaneous skin lesions. Histological examination of an incisional biopsy of a lesion revealed a lobular panniculitis with an inflammatory infiltrate of atypical lymphocytes and evidence of cytophagocytosis consistent with a diagnosis of subcutaneous T-cell lymphoma. The systemic symptoms and skin lesions resolved spontaneously within 3 weeks, only to recur 2 months later, requiring treatment with oral prednisolone. T-cell gene rearrangement studies demonstrated a monoclonal T-cell receptor (gamma-chain) gene rearrangement, further supporting the diagnosis of subcutaneous panniculitic T-cell lymphoma. Treatment with chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) led to remission of symptoms. Four months after completing chemotherapy, the patient remained asymptomatic with a few indurated subcutaneous plaques on the chest. Biopsy of these areas revealed lobular panniculitis, lymphocytic infiltrate without cytological atypia, abundant lipophages and fibrosis and sclerosis consistent with a healing response. He remains well 24 months following chemotherapy.