Background: Cutaneous lymphomas have many morphologic forms and clinical features. Lymphoma presents rarely with a constellation of nodules, panniculitis, and localized lipoatrophy. The histopathologic similarities of lymphoma and connective tissue disease panniculitis may create a diagnostic challenge. Methods: We retrospectively reviewed the case of a 47-year-old man who presented 15.years earlier with recurrent fevers, fatigue, tender subcutaneous nodules, and facial, trunk, and extremity lipoatrophy. Results: Initial biopsy of a cutaneous nodule showed lymphohistiocytic panniculitis without atypical inflammatory cells. Serologic tests showed negative connective tissue serologies, yet the initial clinical impression was most consistent with lupus panniculitis. Initially, the patient was treated with oral prednisone; later, steroid-sparing agents were used with modest improvement evidenced by resolution of his systemic symptoms and stabilization of the cutaneous findings. A decade later, more pronounced facial lipoatrophy, new facial nodules, and posterior thigh pain developed. Laboratory testing showed leukopenia, elevated liver and muscle enzymes, hypertriglyceridemia, and a low level of high-density lipoprotein. Biopsy of a new chin nodule indicated peripheral T-cell lymphoma, whereas an evaluation for systemic malignant involvement was negative. The patient was started on chemotherapy, which resulted in stabilization of the lipoatrophy and decreasing size and frequency of the cutaneous nodules, but the posterior thigh pain persisted. Conclusions: We report a rare case of lymphoma presenting as nodules and profound lipoatrophy, which exemplifies the complexity of lymphomas. Profound lipoatrophy and panniculitis may be an unusual and diagnostically challenging presentation of cutaneous lymphoma.
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