Cutaneous T-cell lymphoma

Abstract

I N 1806, FRENCH dermatologist Baron Jean Louis Alibert’ identified the unique characteristics of the disease process known today as cutaneous T-cell lymphoma (CTCL). He called it “mycosis fungoides” (MF), not because it was a fungus, but because of the mushroom-like nature of the cutaneous lesions found in the advanced stages of the disease. In 1870, Bazin’ described several clinical stages of MF with progression from a premycotic or pretumor stage to placque and nodular tumor development. A fumeur d’emble’e variant was reported by Vidal and Brocq in 18853 and referred to the sudden appearance of de novo tumors. Today, some believe that this event may signify the emergence of a particularly aggressive neoplasm or a sudden decline in host resistance.4 Early in the 20th century, Pautrier recognized the diagnostic importance of the malignant, inflammatory cells that form characteristic band-like aggregates with the superficial dermis and later invade the epidermis as small clusters.5 This cellular debris constitutes the microabcesses that are a hallmark of MF pathology. In 1938, Sezary and Bouvrain6 characterized an unusual syndrome manifested by severe pruritus, generalized exfoliative erythroderma, circulating “monster” blood cells, and peripheral lymphadenopathy with similar atypical cells. Later, ultrastructural analysis of the abnormal cells from the skin, blood, and lymph nodes of patients with Sezary syndrome (SS) revealed their unique hyperconvoluted, cerebriform nuclei.’ Because of the distinct hematologic component, many investigators consider SS to be a leukemic variant of CTCL.’ However, this point is controversial, and the full implication of detecting circulating neoplastic cells has not been fully resolved. In general, CTCL patients with a high per-