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Cutaneous T-cell lymphoma (mycosis fungoides)

Mycosis fungoides is a rare type of skin tumour which often follows a relatively benign course. It presents insidiously with scaly patches and plaques which can look eczematous or psoriasiform. Lesions often appear initially on the buttocks. These lesions may come and go or remain persistent over many years. Patients may well die of unrelated causes. Skin biopsy confirms the diagnosis showing invasion by atypical lymphocytes. T-cell receptor gene rearrangement studies show that there is often a monoclonal expansion of lymphocytes in the skin.

Occasionally the disease can progress to a cutaneous nodular or tumour stage which may be accompanied by systemic organ involvement. In elderly males the disease may progress rarely to an erythrodermic variant accompanied by lymphadenopathy and peripheral blood involvement ('Sezary syndrome'). All patients should be staged at the time of diagnosis to assess for any systemic involvement.

Treatment of Mycosis fungoides

Early cutaneous disease can be left untreated or treated with topical steroids or PUVA. More advanced disease of the skin, or systemic involvement, may require radiotherapy, chemotherapy, immunotherapy or electron beam therapy.



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