Mycosis fungoides

Last Updated: 2022-02-25

Author(s): Anzengruber F., Navarini A.

ICD11: 2B01

MF

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

  • Incidence: 0,4-0,5/100.000/year
  • Occurring from the age of 40
  • Men : women = 2 : 1

  • Main forms
    • Folliculotropic MF
    • Pagetoid reticulosis
    • Granulomatous slack skin
  • Other forms
    • Syringotropic MF
    • Ichtyhyosiform MF
    • Pustular MF
    • Interstitial Mf
    • Vegetative papillomatous MF
    • Bullous MF
    • Hyperkeratotic verrucous MF
    • Erythrodermic mycosis fungoides
    • Palmoplantar MF
    • Poikilodermatitic MF
    • Hyper- and hypopigmented mycosis fungoides

Persistent stimulation from viruses, bacteria or other external influences is suspected as the cause. There is stimulation and proliferation of a lymphocyte clone with chromosomal instability.

  • Mostly the trunk and the flexor sides of the legs and arms are affected
  • Patch stage
    • Erythematous, sharply circumscribed, pityriasiform or psoriasiform, sometimes confluent macules
  • Plaque stage
    • Erythematous to purplish-brownish, lichenified plaques, sometimes associated with alopecia
  • Tumour stage
    • Exophytic, sometimes fungal, erythematous-livid tumours. Often shows erythroderma

  • Anamnesis
    • Duration?
    • Temporal progression?
    • B-symptomatology?
  • Clinic
    • Palpation of all lymph node stations, liver and spleen
  • Laboratory
    • ESR/CRP, differential blood count (often lymphocytosis and eosinophilia), liver enzymes, creatinine, LDH, electrolytes
    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7- cells
      • Immunelectrophoresis if necessary
      • If necessary, HTLV serology (especially for patients from abroad)
      • If necessary, Borrelia serology
    • Frequently elevated
  • Lymphocyte differentiation
    • FACS analysis: CD4+ cells↑, sometimes CD8+ cells↑
  • Sézary -cells (cells with large, indented nucleus of 6-10 μm size and in electron microscopy cerebriform nucleus and glycogen granules in the cytoplasm)?
    • At > 1000 cells/μL à Sézary syndrome
  • Biopsy
    • Immune phenotyping
    • Molecular biology: clonal rearrangment of T-cell receptor genes

Staging see latest SOP.

Patch, plaque stage:

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

Tumour stage:

  • Nodular atypical lymphocytic infiltrates. Epidermotropism and Pautrier microabscesses uncommon

  1. Mycosis Fungoides, in An Illustrated Guide to Skin Lymphoma. Wiley-Blackwell. p. 9-38.
  2. Erratum in Olsen et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:1713-1722. Blood, 2008. 111(9): p. 4830-4830.
  3. Beljaards, R.C., et al., Primary cutaneous T-cell lymphoma: Clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and cd30-positive large cell lymphoma. J. Pathol., 1994. 172(1): p. 53-60.
  4. Beylot-Barry, M., et al., Is bone marrow biopsy necessary in patients with mycosis fungoides and Sezary syndrome? A histological and molecular study at diagnosis and during follow-up. Br J Dermatol, 2005. 152(6): p. 1378-1379.
  5. Bleehen, S.S. and D.N. Slater, (25) Pityriasis lichenoides developing into mycosis fungoides. Br J Dermatol, 1986. 115(s30): p. 69-70.
  6. Boztepe, G., et al., Narrowband ultraviolet B phototherapy to clear and maintain clearance in patients with mycosis fungoides. Journal of the American Academy of Dermatology, 2005. 53(2): p. 242-246.
  7. Bunn, P.A., Systemic Therapy of Cutaneous T-Cell Lymphomas (Mycosis Fungoides and the Sezary Syndrome). Annals of Internal Medicine, 1994. 121(8): p. 592.
  8. de Unamuno Bustos, B., et al., Adult pityriasis lichenoides-like mycosis fungoides: a clinical variant of mycosis fungoides. International Journal of Dermatology, 2014. 53(11): p. 1331-1338.
  9. Demirkesen, C., et al., The clinical features and histopathologic patterns of folliculotropic mycosis fungoides in a series of 38 cases. J Cutan Pathol, 2014. 42(1): p. 22-31.
  10. Díez Recio, E., et al., Topical 5-aminolevulinic acid photodynamic therapy for the treatment of unilesional mycosis fungoides: a report of two cases and review of the literature. International Journal of Dermatology, 2008. 47(4): p. 410-413.
  11. Dummer, R., et al., Aktuelle Aspekte zur Pathogenese von Sézary-Syndrom und Mycosis fungoides. Der Hautarzt, 2001. 52(3): p. 189-192.
  12. Gerami, P., et al., Folliculotropic Mycosis Fungoides. Arch Dermatol, 2008. 144(6).
  13. Gómez Orbaneja, J., et al., Lymphomatoid contact dermatitis A syndrome produced by epicutaneous hypersensitivity with clinical features and a histopathologic picture similar to that of mycosis fungoides. Contact Dermatitis, 1976. 2(3): p. 139-143.
  14. Holcomb, M., M. Duvic, and J. Cutlan, Erythema gyratum repens-like eruptions with large cell transformation in a patient with mycosis fungoides. International Journal of Dermatology, 2011. 51(10): p. 1231-1233.
  15. Jackow, C.M., et al., Follicular mucinosis associated with scarring alopecia, oligoclonal T-cell receptor V? expansion, and Staphylococcus aureus: When does follicular mucinosis become mycosis fungoides? Journal of the American Academy of Dermatology, 1997. 37(5): p. 828-831.
  16. Joly, P., et al., Primary cutaneous medium and large cell lymphomas other than mycosis fungoides. An immunohistological and follow-up study on 54 cases. British Journal of Dermatology, 2006. 132(4): p. 506-512.
  17. Jones, G.W., et al., Total skin electron radiation in the management of mycosis fungoides: Consensus of the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Project Group. Journal of the American Academy of Dermatology, 2002. 47(3): p. 364-370.
  18. Kalayciyan, A., et al., Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not? International Journal of Dermatology, 2004. 43(12): p. 953-956.
  19. Kaye, F.J., et al., A Randomized Trial Comparing Combination Electron-Beam Radiation and Chemotherapy with Topical Therapy in the Initial Treatment of Mycosis Fungoides. New England Journal of Medicine, 1989. 321(26): p. 1784-1790.
  20. Kempf, W., et al., Granulomatous Mycosis Fungoides and Granulomatous Slack Skin. Arch Dermatol, 2008. 144(12).
  21. Kim, Y.H., Management with topical nitrogen mustard in mycosis fungoides. Dermatologic Therapy, 2003. 16(4): p. 288-298.
  22. Kim, Y.H., et al., TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood, 2007. 110(2): p. 479-484.
  23. Klemke, C.-D., et al., Clonal T cell receptor γ-chain gene rearrangement by PCR-based GeneScan analysis in the skin and blood of patients with parapsoriasis and early-stage mycosis fungoides. J. Pathol., 2002. 197(3): p. 348-354.
  24. Lazar, A.P., et al., Parapsoriasis and mycosis fungoides: The Northwestern University experience, 1970 to 1985. Journal of the American Academy of Dermatology, 1989. 21(5): p. 919-923.
  25. Lindae, M.L., Poikilodermatous mycosis fungoides and atrophic large-plaque parapsoriasis exhibit similar abnormalities of T-cell antigen expression. Archives of Dermatology, 1988. 124(3): p. 366-372.
  26. Lipsker, D., The Pigmented and Purpuric Dermatitis and the Many Faces of Mycosis fungoides. Dermatology, 2003. 207(3): p. 246-247.
  27. Nagase, K., et al., CD4/CD8 Double-negative Mycosis Fungoides Mimicking Erythema Gyratum Repens in a Patient with Underlying Lung Cancer. Acta Dermato Venereologica, 2014. 94(1): p. 89-90.
  28. Pimpinelli, N., et al., Defining early mycosis fungoides. Journal of the American Academy of Dermatology, 2005. 53(6): p. 1053-1063.
  29. Prince, H.M., S. Whittaker, and R.T. Hoppe, How I treat mycosis fungoides and Sezary syndrome. Blood, 2009. 114(20): p. 4337-4353.
  30. Resnik, K.S. and E.C. Vonderheid, Home UV phototherapy of early mycosis fungoides: Long-term follow-up observations in thirty-one patients. Journal of the American Academy of Dermatology, 1993. 29(1): p. 73-77.
  31. Rongioletti, F., et al., Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. Journal of Cutaneous Pathology, 2010. 37(1): p. 15-19.
  32. Sanchez, J.L. and B.A. Ackerman, The patch stage of mycosis fungoides Criteria for histologic diagnosis. The American Journal of Dermatopathology, 1979. 1(1): p. 5-26.
  33. Schappell, D.L., Treatment of Advanced Mycosis Fungoides and Sézary Syndrome With Continuous Infusions of Methotrexate Followed by Fluorouracil and Leucovorin Rescue. Arch Dermatol, 1995. 131(3): p. 307.
  34. Shabrawi-Caelen, L.E., et al., Hypopigmented Mycosis Fungoides. The American Journal of Surgical Pathology, 2002. 26(4): p. 450-457.
  35. Talpur, R., R. Bassett, and M. Duvic, Prevalence and treatment of Staphylococcus aureus colonization in patients with mycosis fungoides and Sézary syndrome. Br J Dermatol, 2008. 159(1): p. 105-112.
  36. Trautinger, F., et al., EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. European Journal of Cancer, 2006. 42(8): p. 1014-1030.
  37. uuml, et al., Follicular Mucinosis Associated with Mycosis Fungoides. Dermatology, 1991. 183(1): p. 66-67.
  38. Väkevä, L., et al., A Retrospective Study of the Probability of the Evolution of Parapsoriasis en Plaques into Mycosis Fungoides. Acta Dermato-Venereologica, 2005. 85(4): p. 318-323.
  39. van Doorn, R., E. Scheffer, and R. Willemze, Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis. Arch Dermatol, 2002. 138(2).
  40. van Doorn, R., et al., Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood, 2008. 113(1): p. 127-136.
  41. Whittaker, S.J. and F.M. Foss, Efficacy and tolerability of currently available therapies for the mycosis fungoides and Sezary syndrome variants of cutaneous T-cell lymphoma. Cancer Treatment Reviews, 2007. 33(2): p. 146-160.
  42. Wong, W.-R., et al., Generalized Granuloma annulare Associated with Granulomatous Mycosis fungoides. Dermatology, 2000. 200(1): p. 54-56.
  43. Wood, G.S., et al., Detection of Clonal T-Cell Receptor γ Gene Rearrangements in Early Mycosis Fungoides/Sezary Syndrome by Polymerase Chain Reaction and Denaturing Gradient Gel Electrophoresis (PCR/DGGE). Journal of Investigative Dermatology, 1994. 103(1): p. 34-41.
  44. Zackheim, H.S., Treatment of Mycosis Fungoides With Topical Nitrosourea Compounds. Arch Dermatol, 1972. 106(2): p. 177.
  45. Zackheim, H.S., et al., Percutaneous absorption of I,3-bis (2-chloroethyl)-I-nitrosourea (BCNU, carmustine) in mycosis fungoides. Br J Dermatol, 1977. 97(1): p. 65-67.
  46. Zackheim, H.S., M. Kashani-Sabet, and S. Amin, Topical Corticosteroids for Mycosis Fungoides. Arch Dermatol, 1998. 134(8).
  47. Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.
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