Discussion Mycosis fungoides, Sezary syndrome and related disorders such as lymphomatoid papulosis were described long before the discovery of T cells. With the use of immunohistochemical techniques including T cell assays, the cutaneous lymphomas could be divided according to cell type into T or B cell types. The clinical presentation is a non-specific erythroderma. The skin is diffusely, bright red with scaling and may be accompanied by fever, chills, loss of weight, malaise, insomnia secondary to overwhelming pruritus and poor temperature homeostasis. There may be scaling and fissuring of palms and soles, alopecia, ectropion, nail dystrophy and ankle oedema.
Diagnosis is made by skin biopsy and the finding of abnormal lymphocytes in the blood.
Multiple skin biopsies may be needed because of marked variability of epidermotropism and infiltrate density. Widespread CTCL is commonly found at autopsy but clinically apparent extra-cutaneous disease is detected much less often. The cutaneous affinity of this lymphoma is due to keratinocyte-T cell interaction. It also increases vascular permeability and enhances the effectiveness of other chemoattractants from keratinocytes, such as IL-8. However, many cases of earlier CTCL lesions had no evidence of dominant clonality when analysed by this method.