Mycosis fungoides is a non-Hodgkin's lymphoma that arises from CD4+ T lymphocytes.
The aetiology of mycosis fungoides is unknown, although a variety of risk factors have been proposed, including environmental factors (e.g. occupational exposure, viral infection) and genetic factors (1).
It is a cutaneous lymphoma that characteristically affects middle aged males. It usually begins as an eczematous reaction and proceeds to form plaques, tumours and fungating ulcers. Erythroderma may occur which often is highly pruritic.
Treatment options include topical steroids, topical cytotoxic agents, PUVA and radiotherapy
- visceral involvement may require systemic cytotoxic therapy, although responses
are often short-lived
- systemic therapy options include interferon-alpha, oral retinoids, and chemotherapy drugs
Prognosis relates to extent and type of skin involvement - average survival in the early stage of this disease is at least 10-15 years (2)
Sezary syndrome is the leukaemic presentation of mycosis fungoides (3).
- (1) Morales Suarez-Varela MM et al (2000). Mycosis fungoides: review of epidemiological observations. Dermatol, 201-21-28.
- (2) Dermatology in practice (2001), 9 (2), 14-18.
- (3) Lorincz AI (1996). Cutaneous T-cell lymphoma (mycosis fungoides). Lancet, 347, 871-876.
- (4) Trautinger F, Knobler R, Willemze R, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome. Eur J Cancer. 2006;42(8):1014-1030