Mycosis fungoides

Last Updated: 2021-06-28

Author(s): -


Most frequent primary cutaneous T-cell lymphoma with low malignancy.

  • Incidence: 0,4-0,5/100.000/year.
  • More likely to occur after the age of 40.
  • Men : women = 2 : 1

Main types:

  • Folliculotropic MF
  • Pagetoids Reticulosis
  • Granulomatous slack skin
  • Other types:
    • Syringotropic MF
    • Ichtyhyosiform MF
    • Pustular MF
    • Interstitial MF
    • Vegetable papillomatous MF
    • Bullous MF
    • Hyperkeratotic-versuscous MF
    • Erythrodermic mycosis fungoides
    • Palmoplantare MF
    • Poikilodermatitic MF
    • Hyper- and hypopigmented mycosis fungoides

The cause is suspected to be a persistent stimulation of viruses, bacteria or other external influences. Stimulation and proliferation of a lymphocyte clone with chromosomal instability occurs. 

Especially the trunk as well as the bending sides of the legs and arms are affected.

  • Patch stage:
    • Erythematous, sharply defined, pityriasiform or psoriasiform, partly confluent macules.
  • Plaque stage:
    • Erythematous to violet-brownish, lichenified plaques, partly accompanied by alopecia.
  • Tumor stage:
    • Exophytic, partly fungal, erythematous-livid tumors. Often an erythrodermia shows up.
  • Medical history
    • Duration?
    • Temporal progression
    • B symptoms?
  • Clinical features
    • Palpation of all lymph node stations, liver and spleen.
  • Laboratory
    • BSG/CRP, differential blood count (often lymphocytosis and eosinophilia), liver enzymes, creatinine, LDH, electrolytes.
    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7 cells.
      • If necessary, immune electrophoresis
      • If necessary. HTLV serology (especially for patients from abroad)
      • If necessary, Borrelia serology
    • IgE levels
      • Frequently increased
    • Lymphocyte differentiation
      • FACS analysis: CD4+-cells increased↑, sometimes CD8+ cells increased↑
    • Sézary cells (cells with a large, indented nucleus of 6-10 μm in size and cerebriform nucleus in electron microscopy and glycogen granules in the cytoplasm)?
      • With > 1000 cells/μL the diagnosis of Sézary syndrome
  • Biopsy
    • Immunophenotyping
    • Molecular biology: Clonal rearrangement of T cell receptor genes.

Staging --> consult most current SOP, currently being updated.

Patch, plaque stage:

  • Parakeratosis, possibly acanthosis, exocytosis, atypical lymphocytes with cerebriform nuclei, epidermotropism, Pautrier micro-abscesses and "lining up" at the dermal-epidermal junction.

Tumor stage:

  • Nodular atypical lymphocytic infiltrates. Epidermotropism and pautrier micro-abscesses unusual.
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