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Mycosis fungoides and variants
A group of disorders including the most common forms of cutaneous T-cell lymphomas. The term Mycosis fungoides (MF) is restricted to the classical form characterized by the slow progression of patches, plaques and tumors, and to variants with a similar indolent course.
ORPHA:178566Classification level: Group of disorders
- Synonym(s): -
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: C84.0
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
The annual incidence is estimated at between 1/350,000 and 1/110,000. The male to female ratio is 2:1. MF and its variants typically occur in adults and the elderly.
Besides "classical" MF (Alibert-Bazin type), three distinct variants have been considered as sufficiently distinct to be included in the WHO-EORTC (European organisation for research and treatment of cancer) classification of cutaneous lymphomas: follicular mycosis fungoides, characterized by pilotropic lesions with or without deposition of mucin within the hair follicles; pagetoid reticulosis, characterized by localized lesions with intraepidermal proliferation of neoplastic T-cells usually involving the extremities; and granulomatous slack skin, characterized by areas of pendulous lax skin predominantly in the major skin folds (see these terms). Patches are often hypo- or hyperpigmented in dark-skinned individuals. Pruritus may be present, particularly in cases of folliculotropic MF. Extracutaneous involvement may occur in classical MF.
The etiology remains unknown.
The diagnosis is based on clinical presentation and should be confirmed by a skin biopsy.
Differential diagnoses include inflammatory dermatoses (i.e. atopic dermatitis) in the early stages of MF, and other large cell non-Hodgkin lymphomas (see this term) in later stages.
Management and treatment
Management in the early stages includes mainly PUVA (photochemotherapy), interferon alpha-2a, retinoids, topical chemotherapy, and topical steroids. Total body electron beam irradiation may be used in case of severe forms of the disease. A variety of more aggressive treatments have been used for advanced stages of MF.
MF and its variants are slowly progressive. The prognosis depends on the type of MF but is usually favorable.