Mycosis fungoides
Last Updated: 2023-07-07
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
- Mycosis Fungoides, in An Illustrated Guide to Skin Lymphoma. Wiley-Blackwell. p. 9-38.
- 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.
- 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.
- 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.
- Bleehen, S.S. and D.N. Slater, (25) Pityriasis lichenoides developing into mycosis fungoides. Br J Dermatol, 1986. 115(s30): p. 69-70.
- 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.
- 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.
- 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.
- 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.
- DÃez 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.
- Dummer, R., et al., Aktuelle Aspekte zur Pathogenese von Sézary-Syndrom und Mycosis fungoides. Der Hautarzt, 2001. 52(3): p. 189-192.
- Gerami, P., et al., Folliculotropic Mycosis Fungoides. Arch Dermatol, 2008. 144(6).
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Kempf, W., et al., Granulomatous Mycosis Fungoides and Granulomatous Slack Skin. Arch Dermatol, 2008. 144(12).
- Kim, Y.H., Management with topical nitrogen mustard in mycosis fungoides. Dermatologic Therapy, 2003. 16(4): p. 288-298.
- 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.
- 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.
- 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.
- 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.
- Lipsker, D., The Pigmented and Purpuric Dermatitis and the Many Faces of Mycosis fungoides. Dermatology, 2003. 207(3): p. 246-247.
- 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.
- Pimpinelli, N., et al., Defining early mycosis fungoides. Journal of the American Academy of Dermatology, 2005. 53(6): p. 1053-1063.
- Prince, H.M., S. Whittaker, and R.T. Hoppe, How I treat mycosis fungoides and Sezary syndrome. Blood, 2009. 114(20): p. 4337-4353.
- 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.
- 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.
- 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.
- 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.
- Shabrawi-Caelen, L.E., et al., Hypopigmented Mycosis Fungoides. The American Journal of Surgical Pathology, 2002. 26(4): p. 450-457.
- 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.
- 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.
- uuml, et al., Follicular Mucinosis Associated with Mycosis Fungoides. Dermatology, 1991. 183(1): p. 66-67.
- 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.
- 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).
- van Doorn, R., et al., Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood, 2008. 113(1): p. 127-136.
- 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.
- Wong, W.-R., et al., Generalized Granuloma annulare Associated with Granulomatous Mycosis fungoides. Dermatology, 2000. 200(1): p. 54-56.
- 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.
- Zackheim, H.S., Treatment of Mycosis Fungoides With Topical Nitrosourea Compounds. Arch Dermatol, 1972. 106(2): p. 177.
- 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.
- Zackheim, H.S., M. Kashani-Sabet, and S. Amin, Topical Corticosteroids for Mycosis Fungoides. Arch Dermatol, 1998. 134(8).
- Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.
The connection has been lost. Please stop editing.