Abstract
Mycosis fungoides (MF) is a CD4+ primary cutaneous T-cell lymphoma with a good prognosis. Patients with MF classicly present with pink-to-erythematous patches, well-demarcated plaques, and/or tumors on sun-protected areas, including the flanks, breasts, inner thighs, groin, and buttocks. Cutaneous biopsies of classic MF demonstrate characteristic epidermotropism, “tagging” of atypical T cells along the dermoepidermal junction, and Pautrier microabscesses. There are a number of subtypes of MF with varied presentations, including folliculotropic MF, pagetoid reticulosis, granulomatous slack skin, poikilodermatous MF, and hypopigmented MF. This chapter will discuss the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, and molecular biology of several of the subtypes of MF. It closes with five clinical cases, each representing a different subtype of MF.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Ralfkiaer E, Cerroni L, Sander CA, Smoller BR, Willemze R. Mycosis fungoides. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 296–8.
Bradford PT, Devesa SS, Anderson WF, Toro JR. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood. 2009;113:5064–73.
Willenze R, Jaffees, Burg G, Cerroni L, Berti E, Swerdlow Sh et al. WHO/EORTC classification for Cutaneous Lymphomas. Blood 2005;105:3768–85.
Yazganoglu KD, Topkarci Z, Buyukbabani N, Baykal C. Childhood mycosis fungoides: a report of 20 cases from Turkey. J Eur Acad Dermatol Venereol. 2013;27:295–300.
Kim YH, Liu HL, Mraz-Gernhard S, Varghese A, Hoppe RT. Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Arch Dermatol. 2003;139:857–66.
Marks JG, Miller JJ. In: Marks JG, Miller JJ, editors. Scaling papules, plaques and patches. Lookingbill and Marks’ principles of dermatology. 4th ed. Philadelphia: Saunders Elsevier. p. 134–5.
Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al. Defining early mycosis fungoides. J Am Acad Dermatol. 2005;53:1053–63.
Ralfkiaer E, Willemze R, Whittaker SJ, et al. Sezary syndrome. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 299.
Vergier B, De Muret A, Beylot-barry M, Vaillant L, Ekouevi D, Chene G, et al. Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. Blood. 2000;95:2212–8.
Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, Ferreira 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.
Hwang S, Janik J, Jaffe E, Wilson W. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371:945–57.
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, 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 Ca. Blood. 2007;110:1713–22.
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.
Molina A, Zain J, Arber DA, Angelopolou M, O’Donnell M, Murata-Collins J, et al. Durable clinical, cytogenetic, and molecular remissions after allogeneic hematopoietic cell transplantation for refractory Sezary syndrome and mycosis fungoides. J Clin Oncol. 2005;23:6163–71.
Abbott RA, Whittaker SJ, Morris SL, Russell-Jones R, Hung T, Bashir SJ, et al. Bexarotene therapy for mycosis fungoides and Sézary syndrome. Br J Dermatol. 2009;160:1299–307.
Talpur R, Singh L, Daulat S, Liu P, Seyfer S, Trynosky T, et al. Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res. 2012;18:5051–60.
Kazakov DV, Burg G, Kempf W. Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol. 2004;18:397–415.
Vega F, Luthra R, Medeiros LJ, Dunmire V, Lee S-J, Duvic M, et al. Clonal heterogeneity in mycosis fungoides and its relationship to clinical course. Blood. 2002;100:3369–73.
Van Doorn R, van Kester MS, Dijkman R, Vermeer MH, Mulder AA, Szuhai K, et al. Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood. 2009;113:127–36.
Carbone A, Bernardini L, Valenzano F, Bottillo I, De Simone C, Capizzi R, et al. Array-based comparative genomic hybridization in early-stage mycosis fungoides: recurrent deletion of tumor suppressor genes BCL7A, SMAC/DIABLO, and RHOF. Genes Chromosomes Cancer. 2008;47:1067–75.
Fischer TC, Gellrich S, Muche JM, Sherev T, Audring H, Neitzel H, et al. Genomic aberrations and survival in cutaneous T cell lymphomas. J Invest Dermatol. 2004;122:579–86.
Gallardo F, Esteller M, Pujol RM, Costa C, Estrach T, Servitje O. Methylation status of the p15, p16 and MGMT promoter genes in primary cutaneous T-cell lymphomas. Haematologica. 2004;89:1401–3.
Mao X, Lillington D, Scarisbrick JJ, Mitchell T, Czepulkowski B, Russell-Jones R, et al. Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides. Br J Dermatol. 2002;147:464–75.
Navas IC, Ortiz-Romero PL, Villuendas R, Martínez P, García C, Gómez E, et al. p16(INK4a) gene alterations are frequent in lesions of mycosis fungoides. Am J Pathol. 2000;156:1565–72.
Prochazkova M, Chevret E, Mainhaguiet G, Sobotka J, Vergier B, Belaud-Rotureau M-A, et al. Common chromosomal abnormalities in mycosis fungoides transformation. Genes Chromosomes Cancer. 2007;46:828–38.
Scarisbrick JJ, Mitchell TJ, Calonje E, Orchard G, Russell-Jones R, Whittaker SJ. Microsatellite instability is associated with hypermethylation of the hMLH1 gene and reduced gene expression in mycosis fungoides. J Invest Dermatol. 2003;121:894–901.
Van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis. Arch Dermatol. 2002;138:191–8.
Lehman J, Cook-Norris RH, Weed BR, Weenig RH, Gibson LE, Weaver AL, et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol. 2010;146:607–13.
Tannous Z, Baldassano MF, Li VW, Kvedar J, Duncan LM. Syringolymphoid hyperplasia and follicular mucinosis in a patient with cutaneous T-cell lymphoma. J Am Acad Dermatol. 1999;41:303–8.
Haghighi B, Smoller BR, LeBoit PE, Warnke RA, Sander CA, Kohler S. Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular, and clinicopathologic study. Mod Pathol. 2000;13:502–10.
Steffen C. Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis. Am J Dermatopathol. 2005;27:68–85.
Kempf W, Ostheeren-michaelis S, Paulli M, Lucioni M, Wechsler J, Audring H, et al. Granulomatous mycosis fungoides and granulomatous slack skin. Arch Dermatol. 2008;144:1609–17.
Kempf W, Kazakov DV, Palmedo G, Fraitag S, Schaerer L, Kutzner H. Pityriasis lichenoides et varioliformis acuta with numerous CD30(+) cells: a variant mimicking lymphomatoid papulosis and other cutaneous lymphomas. A clinicopathologic, immunohistochemical, and molecular biological study of 13 cases. Am J Surg Pathol. 2012;36:1021–9.
Ardigó M, Borroni G, Muscardin L, Kerl H, Cerroni L. Hypopigmented mycosis fungoides in Caucasian patients: a clinicopathologic study of 7 cases. J Am Acad Dermatol. 2003;49:264–70.
Pavlovsky L, Mimouni D, Amitay-Laish I, Feinmesser M, David M, Hodak E. Hyperpigmented mycosis fungoides: an unusual variant of cutaneous T-cell lymphoma with a frequent CD8+ phenotype. J Am Acad Dermatol. 2012;67:69–75. Elsevier Inc.
Pileri A, Facchetti F, Rütten A, Zumiani G, Boi S, Fink-Puches R, et al. Syringotropic mycosis fungoides: a rare variant of the disease with peculiar clinicopathologic features. Am J Surg Pathol. 2011;35:100–9.
Scarabello A, Leinweber B. Cutaneous lymphomas with prominent granulomatous reaction: a potential pitfall in the histopathologic diagnosis of cutaneous T-and B-cell lymphomas. Am J Surg Pathol. 2002;26:1259–68.
Nashan D, Faulhaber D, Ständer S, Luger TA, Stadler R. Mycosis fungoides: a dermatological masquerader. Br J Dermatol. 2007;156:1–10.
Johnson MAN, Armstrong AW. Clinical and histologic diagnostic guidelines for psoriasis: a critical review. Clin Rev Allergy Immunol. 2013;44:166–72.
Griffiths C, Barker J. Pathogenesis and clinical features of psoriasis. Lancet. 2007;270:263–71.
Fung MA. “Epidermotropism” vs. “exocytosis” of lymphocytes 101: definition of terms. J Cutan Pathol. 2010;37:525–9.
Brady SP, Magro CM, Diaz-Cano SJ, Wolfe HJ. Analysis of clonality of atypical cutaneous lymphoid infiltrates associated with drug therapy by PCR/DGGE. Hum Pathol. 1999;30:130–6.
Guitart J, Magro C. Cutaneous T-cell lymphoid dyscrasia. Arch Dermatol. 2014;143:921–32.
Matutes E. Adult T-cell leukaemia/lymphoma. J Clin Pathol. 2007;60:1373–7.
Yamaguchi T, Ohshima K, Karube K, Tutiya T, Kawano R, Suefuji H, et al. Clinicopathological features of cutaneous lesions of adult T-cell leukaemia/lymphoma. Br J Dermatol. 2005;152:76–81.
Jaffe E, Cossman J, Blattner W, Robert-Guroff M, Blayney D, Gallo RC, et al. The pathologic spectrum of adult T-cell leukemia/lymphoma in the United States. Am J Surg Pathol. 1984;8:263–75.
Tsukasaki K, Hermine O, Bazarbachi A, Ratner L, Ramos JC, Harrington W, et al. Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting. J Clin Oncol. 2009;27:453–9.
Kunishige JH, McDonald H, Alvarez G, Johnson M, Prieto V, Duvic M. Lymphomatoid papulosis and associated lymphomas: a retrospective case series of 84 patients. Clin Exp Dermatol. 2009;34:576–81.
Cerroni L, Fink-Puches R, Back B, Kerl H. Follicular mucinosis: a critical reappraisal of clinicopathologic features and associated with mycosis fungoides and Sezary syndrome. Arch Dermatol. 2002;138:182–9.
Madani S, Shapiro J. Alopecia areata update. J Am Acad Dermatol. 2000;42:549–66.
Zhang J-A, Yu J-B. Hypopigmented mycosis fungoides in a Chinese woman. Indian J Dermatol. 2013;58:161.
Mendez-Tovar LJ. Pathogenesis of dermatophytosis and tinea versicolor. Clin Dermatol. 2010;28:185–9.
Blessmann Weber M, Sponchiado de Avila LG, Albaneze R, Magalhães de Oliveira OL, Sudhaus BD, Cestari TF. Pityriasis alba: a study of pathogenic factors. J Eur Acad Dermatol Venereol. 2002;16:463–8.
Vargas-Ocampo F. Pityriasis alba: a histologic study. Int J Dermatol. 1993;32:870–3.
Nofal A, Salah E. Acquired poikiloderma: proposed classification and diagnostic approach. J Am Acad Dermatol. 2013;69:e129–40.
Magro CM, Schaefer JT, Crowson AN, Li J, Morrison C. Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles. Am J Clin Pathol. 2007;128:218–29.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Goyal, A. et al. (2015). Mycosis Fungoides. In: Carter, J., Goyal, A., McDivitt Duncan, L. (eds) Atlas of Cutaneous Lymphomas. Springer, Cham. https://doi.org/10.1007/978-3-319-17217-0_5
Download citation
DOI: https://doi.org/10.1007/978-3-319-17217-0_5
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-17216-3
Online ISBN: 978-3-319-17217-0
eBook Packages: MedicineMedicine (R0)