Cutaneous T-Cell Lymphoma (Mycosis Fungoides)

Mycosis fungoides is the most common variant of primary cutaneous T-cell lymphoma (CTCL), a T-helper cell lymphoma of the skin. The cause is unknown. It has a male : female ratio of 2 : 1, with most patients diagnosed in their fifties and sixties.

CTCL can develop from patch stage to limited or extensive plaque stage and then tumour or nodular stage (Figure 36.1). The term parapsoriasis is used to describe a very early pre-diagnostic phase of CTCL. Multiple biopsies may be required over many years prior to definitive diagnosis.

Patients with CTCL present with ill-defined, red to pink scaly patches with atrophy and telangiectasia. The plaque stage consists of more red to brown elevated patches and plaques in the bathing trunk area affecting the buttocks, hip and upper thighs. Nodules that can ulcerate develop in the tumour stage. The majority have an indolent, slowly evolving disease but any form of CTCL may eventually invade the lymph nodes, peripheral blood and internal organs with a poor prognosis.

Sézary syndrome is a leukaemic form that can evolve from classic CTCL or develop with erythroderma (generalised scaling and exfoliative dermatitis). Pruritus and lymph node enlargement are common in Sézary syndrome.

Investigations include physical examination for lymph nodes and hepatosplenomegaly with a peripheral blood smear examination for Sézary cells.

T-cell receptor gene rearrangement (polymerase chain reaction and/or molecular studies) can be useful to identify monoclonal proliferation of T-cell clones in the skin. Renal and liver function, biopsy of enlarged lymph nodes, chest X-ray, CT scan and bone marrow biopsy may be required.

Histology typically shows superficial and deep band-like perivascular lymphocytic infiltrate with collections of lymphocytes (Pautrier’s micro-abscesses) with thickened epidermis. The infiltrate is mixed with lymphocytes, eosinophils and plasma cells. Lymphocytes can be atypical with a hyper-convoluted or cerebriform nucleus (Sézary cells).

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