Myocosis fungoides--an update on a non-mycotic disease

Mycosis fungoides was first described in 1806 by the French physician Jean Louis Alibert in a patient whose skin lesions developed into mushroom-like tumors. Though it is not an infectious disease, it was termed mycosis fungoides (MF) due to its fungating appearance. In 1870, Bazin further described MF, proposing the three classical stages of the cutaneous disease: patch, plaque, and tumor. The term cutaneous T-cell lymphoma (CTCL) was first utilized in 1975 by Lutzner et al to describe a group of malignant infiltrative disorders of the skin including MF and Sézary syndrome. CTCLs comprise a spectrum of extranodal non-Hodgkin's lymphomas that are characterized by primary cutaneous involvement of a dominant clonal T-cell. As molecular biology and immunohistochemistry techniques have become more developed, CTCL has become understood to be a heterogeneous assembly of disorders that vary with regards to clinical course, histopathology, therapeutic considerations, and prognosis. MF, a low-grade lymphoproliferative disorder, is the most common type of CTCL, comprising 54% of CTCLs. It is a rare, extranodal, non-Hodgkin's lymphoma and is an epidermotropic neoplasm composed of CD4+ (helper) lymphocytes Sézary syndrome is a related leukemic subtype of CTCL that presents with diffuse skin involvement as well as circulating tumor cells in the peripheral blood.