When mycosis fungoides seems not to be within the spectrum of clinical and histopathological differential diagnoses


Published: 7 May 2024
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Authors

The most prevalent primary cutaneous T-cell lymphoma, mycosis fungoides (MF), is characterized by the development of plaques and nodules after an erythematous patchy phase that is non-specific. An infiltrate of atypical small- to medium-sized cerebriform lymphocytes in the superficial dermis, with variable epidermotropism, is the histopathological hallmark of the disease. In more advanced stages of the illness, large-cell transformation may be seen. Early diagnosis of MF can be very challenging based only on histopathologic or clinical findings, so it is critical to have a clinical-pathological correlation. Many atypical variants of MF that deviate from the classic Alibert-Bazin presentation of the disease have been described over the past 30 years, sometimes with different prognostic and therapeutic implications. Clinically or histopathologically, they can mimic a wide range of benign inflammatory skin disorders. To make a conclusive diagnosis in these cases, it is recommended to take multiple biopsies from various lesions and to carefully correlate the clinical and pathological findings. We have outlined the various facets of the illness in this review, positioning MF as a “great imitator”, with an emphasis on the more recently identified variations, differential diagnosis, and its benign mimics.


Elder DE, Massi D, Scolyer RA WR. WHO Classification of Skin Tumours. 4th Edition. IARC Press; 2018.

Muñoz-González H, Molina-Ruiz AM, Requena L. Clinicopathologic variants of mycosis fungoides. Actas Dermo-Sifiliográficas 2017;108:192-208. DOI: https://doi.org/10.1016/j.adengl.2017.02.010

Fauconneau A, Pham-Ledard A, Cappellen D, et al. Assessment of diagnostic criteria between primary cutaneous anaplastic large-cell lymphoma and CD30-rich transformed mycosis fungoides; A study of 66 cases. Br J Dermatol 2015;172:1547-54. DOI: https://doi.org/10.1111/bjd.13690

Massone C, Kodama K, Kerl H, Cerroni L. Histopathologic features of early (patch) lesions of mycosis fungoides: A morphologic study on 745 biopsy specimens from 427 patients. Am J Surg Pathol 2005;29:550-60. DOI: https://doi.org/10.1097/01.pas.0000153121.57515.c6

Massone C, Crisman G, Kerl H, Cerroni L. The prognosis of early mycosis fungoides is not influenced by phenotype and Tcell clonality. Br J Dermatol 2008;159:881-6. DOI: https://doi.org/10.1111/j.1365-2133.2008.08761.x

Kirsch IR, Watanabe R, O’Malley JT, et al. TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL. Sci Transl Med 2015;7:308ra158. DOI: https://doi.org/10.1126/scitranslmed.aaa9122

Harmon CB Witzig TE, Katzmann JA, Pittelkow MR. Detection of circulating T cells with CD4+CD7- immunophenotype in patients with benign and malignant lymphoproliferative dermatoses. J Am Acad Dermatol 1996;35:404-10. DOI: https://doi.org/10.1016/S0190-9622(96)90605-2

Miyashiro D, Sanches JA. Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management. Front Oncol 2023;13:1141108. DOI: https://doi.org/10.3389/fonc.2023.1141108

Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. World Heal Organ Calssification Tumours Haematop Lymphoid Tissue. Lyon, France: 2017.

Mitteldorf C, Stadler R, Sander CA, Kempf W. Folliculotropic mycosis fungoides. J Dtsch Dermatol Ges 2018;16:543-57. DOI: https://doi.org/10.1111/ddg.13514

Hodak E, Amitay-Laish I, Atzmony L, et al. New insights into folliculotropic mycosis fungoides (FMF): a single-center experience. J Am Acad Dermatol 2016;75:347-355. DOI: https://doi.org/10.1016/j.jaad.2016.03.009

Van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol 2002;138:191-8. DOI: https://doi.org/10.1001/archderm.138.2.191

Gerami P, Rosen S, Kuzel T, et al. Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008;144:738-46. DOI: https://doi.org/10.1001/archderm.144.6.738

Lehman JS, Cook-Norris RH, et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010;146:607-13. DOI: https://doi.org/10.1001/archdermatol.2010.101

Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: Validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol 2010;28:4730-9. DOI: https://doi.org/10.1200/JCO.2009.27.7665

Benner MF, Jansen PM, Vermeer MH, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood 2012;119:1643-9. DOI: https://doi.org/10.1182/blood-2011-08-376319

Mantaka P, Helsing P, Gjersvik P, et al. Clinical and histopathological features of folliculotropic mycosis fungoides: a norwegian patient series. Acta Derm Venereol 2013;93:325-9. DOI: https://doi.org/10.2340/00015555-1476

Benton EC, Crichton S, Talpur R, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer 2013;49:2859-68. DOI: https://doi.org/10.1016/j.ejca.2013.04.018

Demirkesen C, Esirgen G, Engin B, et al. The clinical features and histopathologic patterns of folliculotropic mycosis fungoides in a series of 38 cases. J Cutan Pathol 2015;42:22-31. DOI: https://doi.org/10.1111/cup.12423

Muniesa C, Estrach T, Pujol RM, et al. Folliculotropic mycosis fungoides: Clinicopathological features and outcome in a series of 20 cases. J Am Acad Dermatol 2010;62:418-26. DOI: https://doi.org/10.1016/j.jaad.2009.03.014

Hodak E, Amitay-Laish I, Feinmesser M, et al. Juvenile mycosis fungoides: Cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol 2014;70:993-1001. DOI: https://doi.org/10.1016/j.jaad.2013.12.029

Tomasini C, Kempf W, Novelli M, et al. Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases. J Cutan Pathol 2015;42:164-72. DOI: https://doi.org/10.1111/cup.12399

Van Santen S, Roach REJ, Van Doorn R, et al. Clinical staging and prognostic factors in Folliculotropic Mycosis Fungoides. JAMA Dermatol 2016;152:992-1000. DOI: https://doi.org/10.1001/jamadermatol.2016.1597

Tomasini C, Michelerio A, Quaglino P. Spiky/keratosispilaris-like early follicular mycosis fungoides: A clinicopathologic study of 20 cases with extended follow-up. J Cutan Pathol 2021;48:1124-32. DOI: https://doi.org/10.1111/cup.14002

Caccetta TP, Dessauvagie B, McCallum D, Kumarasinghe SP. Multiple minute digitate hyperkeratosis: A proposed algorithm for the digitate keratoses. J Am Acad Dermatol 2012;67:e49-55. DOI: https://doi.org/10.1016/j.jaad.2010.07.023

Roca-Ginés J, Alfaro-Cervelló C, Sánchez-Arraez J, et al. Folliculotropic mycosis fungoides with flexural eruptive cysts and comedones in a 14-year-old boy. Am J Dermatopathol 2021;43:656-8. DOI: https://doi.org/10.1097/DAD.0000000000001958

Amin SM, Tan T, Guitart J, et al. CD8+ mycosis fungoides clinically masquerading as alopecia areata. J Cutan Pathol 2016;43:1179-82. DOI: https://doi.org/10.1111/cup.12805

Shamim H, Riemer C, Weenig R, et al. Acneiform presentations of folliculotropic mycosis fungoides. Am J Dermatopathol 2021;43:85-92. DOI: https://doi.org/10.1097/DAD.0000000000001698

André R, Ram-Wolff C, Bagot M. Clinical characteristics of mycosis fungoides palmaris et plantaris: two cases and a systematic literature review. Eur J Dermatol 2022;32:421-3.

Diehl J, Sarantopoulos GP, Chiu MW. Dyshidrotic mycosis fungoides. J Cutan Pathol 2011;38:590-2. DOI: https://doi.org/10.1111/j.1600-0560.2011.01682.x

Jakob T, Tiemann M, Kuwert C, et al. Dyshidrotic cutaneous T-cell lymphoma. J Am Acad Dermatol 1996;34:295-7. DOI: https://doi.org/10.1016/S0190-9622(96)80139-3

Juan-Carpena G, Palazón-Cabanes JC, Blanes-Martínez M. Successful treatment of palmoplantar dyshidrotic lesions of mycosis fungoides with conventional and daylight photodynamic therapy. J Dtsch Dermatol Ges 2021; DOI: https://doi.org/10.1111/ddg.14403

Leinweber B, Chott A, Kerl H, Cerroni L. Epidermotropic precursor T-cell lymphoma with highly aggressive clinical behavior simulating localized pagetoid reticulosis. Am J Dermatopathol 2007;29:392-4. DOI: https://doi.org/10.1097/DAD.0b013e31812f51b7

Li JY, Pulitzer MP, Myskowski PL, et al. A case-control study of clinicopathologic features, prognosis, and therapeutic responses in patients with granulomatous mycosis fungoides. J Am Acad Dermatol 2013;69:366-74. DOI: https://doi.org/10.1016/j.jaad.2013.03.036

Kempf W, Ostheeren-Michaelis S, Paulli M, et al. Granulomatous mycosis fungoides and granulomatous slack skin: A multicenter study of the cutaneous lymphoma histopathology task force group of the European Organization for Research and Treatment of Cancer (EORTC). Arch Dermatol 2008;144:160-17. DOI: https://doi.org/10.1001/archdermatol.2008.46

Van Doorn R, Van Kester MS, Dijkman R, et al. Oncogenomic analysis of mycosis fungoides reveals major differences with Sézary syndrome. Blood 2009;113:127:36. DOI: https://doi.org/10.1182/blood-2008-04-153031

Laharanne E, Oumouhou N, Bonnet F, et al. Genome-wide analysis of cutaneous T-cell lymphomas identifies three clinically relevant classes. J Invest Dermatol 2010;130:1707-18. DOI: https://doi.org/10.1038/jid.2010.8

Hurabielle C, Michel L, Ram-Wolff C, et al. Expression of Sézary biomarkers in the blood of patients with erythrodermic mycosis fungoides. J Invest Dermatol 2016;136:317-20. DOI: https://doi.org/10.1038/JID.2015.360

Rodney IJ, Kindred C, Angra K, et al. Hypopigmented mycosis fungoides: a retrospective clinicohistopathologic study. J Eur Acad Dermatol Venereol 2017;31:808-14. DOI: https://doi.org/10.1111/jdv.13843

Furlan FC, De Paula Pereira BA, Da Silva LF, Sanches JA. Loss of melanocytes in hypopigmented mycosis fungoides: a study of 18 patients. J Cutan Pathol 2014;41:101-7. DOI: https://doi.org/10.1111/cup.12262

Pavlovsky L, Mimouni D, Amitay-Laish I, et al. Hyperpigmented mycosis fungoides: An unusual variant of cutaneous T cell lymphoma with a frequent CD8+ phenotype. J Am Acad Dermatol 2012;67:69-75. DOI: https://doi.org/10.1016/j.jaad.2011.06.023

Kodama K, Fink-Puches R, Massone C, et al. Papular mycosis fungoides: A new clinical variant of early mycosis fungoides. J Am Acad Dermatol 2005;52:694:8. DOI: https://doi.org/10.1016/j.jaad.2004.12.018

Di Prete, M., Michelerio, A., Lora, V., Tomasini, C. F., & Cota, C. (2024). When mycosis fungoides seems not to be within the spectrum of clinical and histopathological differential diagnoses. Dermatology Reports, 16(s2). https://doi.org/10.4081/dr.2024.10008

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