Abstract:
A 78-year-old Caucasian male with a medical history of atrial fibrillation, type 2 diabetes mellitus, hypertension, and peripheral vascular disease presented with a generalized body rash that persisted for over a year with no improvement despite various topical treatments. He had no history of weight loss, anorexia, fever, or other B symptoms except for chronic stable fatigue. His physical examination on presentation was essentially normal other than generalized erythematous rash over chest, trunk, extremities. No organomegaly or palpable enlarged lymph nodes were appreciated. He had a shave biopsy of skin lesion which revealed mycosis fungoides. Bone marrow biopsy revealed normocellular bone marrow for age with trilineage hematopoiesis but revealed low-level involvement by Sezary syndrome. He later had a PET scan which revealed hypermetabolic lymphadenopathy in the mediastinum, bilateral hillier and upper abdominal region. He was started on Bexarotene and topical steroids for treatment of Sezary syndrome. He remains clinically stable with no worsening skin rash or new B symptoms.

Discussion:
Mycosis Fungoides (MF) is the most common cutaneous T cell lymphoma. Most patients have an indolent course. Sezary Syndrome is the leukemic variant of cutaneous T cell lymphoma (CTTCL) and is related to mycosis fungoides. However, Sezary syndrome is rare, accounting for less than 5% of CTCL and affects older individuals. Atypical T cells (Sézary cells) in skin causes diffuse erythema (erythroderma) and significant blood involvement with abnormal T cells (>1000 abnormal cells/uL) defined as Sézary cells by cytopathologic assessment or flow cytometry. Treatment of sezary syndrome is aimed at controlling systemic disease and skin involvement. Depending on patient’s performance status, comorbidities, severity of involvement, systemic treatment options include Bexarotene, Mogamulizumab, Romidepsin, Methotrexate, Extracorporeal photopheresis, Pralatrexate, Pembrolizumab, Alemtuzumab, Brentuximab, Vorinostat, Interferon Alfa, Retinoids. Skin directed treatment options include UVB, topical steroids, nitrogen mustard, total skin electron beam therapy. Despite several treatment options, most treatments do not result in long durable responses. Aggressive disease course is typically seen in patients with Late stage, elevated LDH, folliculotropic variant and large cell transformation. Such patients should be considered for allogeneic stem cell transplant. Median survival for high risk disease is approximately 34 months. Prompt diagnosis and evaluation of possible symptoms including skin rash is vital in appropriate and timely treatment in this difficult to treat disease. Internists should have a low threshold for evaluating persistent skin rash.

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