Cutaneous T-Cell Lymphoma Differential Diagnoses
- Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD more...
Burg G, Kempf W. Etiology and pathogenesis of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005.
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005;105(10):3768-85. [Medline].
Burg G, Kempf W, Cozzio A, et al. Cutaneous malignant lymphomas: update 2006. J Dtsch Dermatol Ges. Nov 2006;4(11):914-33. [Medline].
Khamaysi Z, Ben-Arieh Y, Izhak OB, Epelbaum R, Dann EJ, Bergman R. The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center. Am J Dermatopathol. Feb 2008;30(1):37-44. [Medline].
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol. Aug 1999;155(2):483-92. [Medline].
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. Dec 2008;144(12):1609-17. [Medline].
Vonderheid EC, Bernengo MG, Burg G, et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol. Jan 2002;46(1):95-106. [Medline].
Setoyama M, Katahira Y, Kanzaki T. Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought. J Dermatol. Dec 1999;26(12):785-90. [Medline].
Weenig RH, Ng CS, Perniciaro C. Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature. Am J Dermatopathol. Jun 2001;23(3):206-15. [Medline].
Hoque SR, Child FJ, Whittaker SJ, et al. Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol. Mar 2003;148(3):516-25. [Medline].
Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients. Am J Surg Pathol. Jun 2004;28(6):719-35. [Medline].
Burg G, Dummer R, Wilhelm M, et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med. Oct 10 1991;325(15):1078-81. [Medline].
El Shabrawi-Caelen L, Cerroni L, Kerl H. The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Semin Cutan Med Surg. Jun 2000;19(2):118-23. [Medline].
Miyamoto T, Yoshino T, Takehisa T, Hagari Y, Mihara M. Cutaneous presentation of nasal/nasal type T/NK cell lymphoma: clinicopathological findings of four cases. Br J Dermatol. Sep 1998;139(3):481-7. [Medline].
Arnulf B, Copie-Bergman C, Delfau-Larue MH, et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. Mar 1 1998;91(5):1723-31. [Medline].
Jaffe ES, Krenacs L, Raffeld M. Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol. Jul 2003;40(3):175-84. [Medline].
Bekkenk MW, Vermeer MH, Jansen PM, et al. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood. Sep 15 2003;102(6):2213-9. [Medline].
Alsaleh QA, Nanda A, Al-Ajmi H, et al. Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991-2006. Int J Dermatol. Dec 2010;49(12):1393-8. [Medline].
Pope E, Weitzman S, Ngan B, et al. Mycosis fungoides in the pediatric population: report from an international childhood registry of cutaneous lymphoma. J Cutan Med Surg. Jan-Feb 2010;14(1):1-6. [Medline].
Herrmann JL, Hughey LC. Recognizing large-cell transformation of mycosis fungoides. J Am Acad Dermatol. Jan 18 2012;[Medline].
Shimauchi T, Sugita K, Nakamura M, Tokura Y. Leukaemic cutaneous T-cell lymphoma-manifesting papuloerythroderma with CD3(-) CD4(+) phenotype. Acta Derm Venereol. 2010;90(1):68-72. [Medline].
Roeschm A, Schleyer V, Landthaler M, Vogt T. Follicular mycosis fungoides: variability of a rare entity. Skinmed. Jan-Feb 2005;4(1):12-7. [Medline].
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. Mar 2010;62(3):418-26. [Medline].
Pileri A, Facchetti F, Rutten A, et al. Syringotropic mycosis fungoides: a rare variant of the disease with peculiar clinicopathologic features. Am J Surg Pathol. Jan 2011;35(1):100-9. [Medline].
Kohler S. Extranodal NK/T-cell lymphoma, nasal type. In: LeBoit P, Burg G, Weedon D, Lyon SA. WHO Books: Tumors of the Skin. Geneva: World Health Organization IARC. X. 2006:191-2.
Clarijs M, Poot F, Laka A, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology. 2003;206(4):393-7. [Medline].
Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol. Jul 2000;136(7):889-96. [Medline].
Torok L, Gurbity TP, Kirschner A, Krenacs L. Panniculitis-like T-cell lymphoma clinically manifested as alopecia. Br J Dermatol. Oct 2002;147(4):785-8. [Medline].
Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. May 1 2003;101(9):3407-12. [Medline].
Santucci M, Pimpinelli N, Massi D, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer. Feb 1 2003;97(3):610-27. [Medline].
von den Driesch P, Coors EA. Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a report of 3 cases stable for years. J Am Acad Dermatol. Apr 2002;46(4):531-5. [Medline].
Barrionuevo C, Anderson VM, Zevallos-Giampietri E, et al. Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. Appl Immunohistochem Mol Morphol. Mar 2002;10(1):7-14. [Medline].
Chen HH, Hsiao CH, Chiu HC. Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma. Br J Dermatol. Sep 2002;147(3):587-91. [Medline].
Bi MY, Curry JL, Christiano AM, Hordinsky MK, et al. The spectrum of hair loss in patients with mycosis fungoides and Sezary syndrome. J Am Acad Dermatol. Jan 2011;64(1):53-63. [Medline].
Livingstone I, Ramamurthi S, Drummond S, Kemp E, Roberts F. Corneal perforation due to limbal involvement in Sézary syndrome. Graefes Arch Clin Exp Ophthalmol. Jan 21 2011;[Medline].
Gutte R, Kharkar V, Mahajan S, Chikhalkar S, Khopkar U. Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy. Indian J Dermatol Venereol Leprol. Nov-Dec 2010;76(6):686-90. [Medline].
Karube K, Aoki R, Nomura Y, et al. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas: analysis of 490 cases. Pathol Int. Feb 2008;58(2):89-97. [Medline].
Russell-Jones R. Diagnosing erythrodermic cutaneous T-cell lymphoma. Br J Dermatol. Jul 2005;153(1):1-5. [Medline].
Lange-Asschenfeldt S, Babilli J, Beyer M, Ri´us-Diaz F, Gonza´lez S, Stockfleth E, et al. Consistency and distribution of reflectance confocal microscopy features for diagnosis of cutaneous T cell lymphoma. J Biomed Opt. Jan 2012;17(1):016001. [Medline].
Petrella T, Comeau MR, Maynadie M, et al. 'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol. Jul 2002;26(7):852-62. [Medline].
Smoller BR, Santucci M, Wood GS, Whittaker SJ. Histopathology and genetics of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Dec 2003;17(6):1277-311. [Medline].
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. May 2000;13(5):502-10. [Medline].
Mao X, Lillington D, Scarisbrick JJ, et al. Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides. Br J Dermatol. Sep 2002;147(3):464-75. [Medline].
Burg G, Zwingers T, Staegemeir E, Santucci M. Interrater and intrarater variabilities in the evaluation of cutaneous lymphoproliferative T-cell infiltrates. EORTC-Cutaneous Lymphoma Project Group. Dermatol Clin. Apr 1994;12(2):311-4. [Medline].
Santucci M, Biggeri A, Feller AC, Massi D, Burg G. Efficacy of histologic criteria for diagnosing early mycosis fungoides: an EORTC cutaneous lymphoma study group investigation. European Organization for Research and Treatment of Cancer. Am J Surg Pathol. Jan 2000;24(1):40-50. [Medline].
Santucci M, Smoller B. ISCL study on early diagnosis of mycosis fungoides. (in preparation).
Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. Dec 2005;53(6):1053-63. [Medline].
Zelger B, Sepp N, Weyrer K, Grünewald K, Zelger B. Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides?. Br J Dermatol. Jun 1994;130(6):765-9. [Medline].
Gerami P, Guitart J. Basaloid folliculolymphoid hyperplasia: a distinctive finding in follicular mycosis fungoides. J Cutan Pathol. Dec 2007;34 Suppl 1:29-32. [Medline].
Imai S, Burg G, Braun-Falco O. Mycosis fungoides and Sezary's syndrome show distinct histomorphological features. Dermatologica. 1986;173(3):131-5. [Medline].
Kamarashev J, Burg G, Kempf W, Hess Schmid M, Dummer R. Comparative analysis of histological and immunohistological features in mycosis fungoides and Sézary syndrome. J Cutan Pathol. Sep 1998;25(8):407-12. [Medline].
Sibaud V, Beylot-Barry M, Thiebaut R, et al. Bone marrow histopathologic and molecular staging in epidermotropic T-cell lymphomas. Am J Clin Pathol. Mar 2003;119(3):414-23. [Medline].
Takeshita M, Okamura S, Oshiro Y, et al. Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan. Hum Pathol. Feb 2004;35(2):231-9. [Medline].
Beljaards RC, Meijer CJ, Van der Putte SC, 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. Jan 1994;172(1):53-60. [Medline].
Chan JK, Sin VC, Wong KF, et al. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. Jun 15 1997;89(12):4501-13. [Medline].
Natkunam Y, Smoller BR, Zehnder JL, Dorfman RF, Warnke RA. Aggressive cutaneous NK and NK-like T-cell lymphomas: clinicopathologic, immunohistochemical, and molecular analyses of 12 cases. Am J Surg Pathol. May 1999;23(5):571-81. [Medline].
de Wolf-Peeters C, Achten R. gammadelta T-cell lymphomas: a homogeneous entity?. Histopathology. Apr 2000;36(4):294-305. [Medline].
Jones D, Vega F, Sarris AH, Medeiros LJ. CD4-CD8-"Double-negative" cutaneous T-cell lymphomas share common histologic features and an aggressive clinical course. Am J Surg Pathol. Feb 2002;26(2):225-31. [Medline].
Olsen EA, Rook AH, Zic J, et al. Sezary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. Feb 2011;64(2):352-404. [Medline].
Ahern K, Gilmore ES, Poligone B. Pruritus in cutaneous T-cell lymphoma: A review. J Am Acad Dermatol. Jan 26 2012;[Medline].
Knobler E. Current management strategies for cutaneous T-cell lymphoma. Clin Dermatol. May-Jun 2004;22(3):197-208. [Medline].
Kanokrungsee S, Rajatanavin N, Rutnin S, Vachiramon V. Efficacy of narrowband ultraviolet B twice weekly for hypopigmented mycosis fungoides in Asians. Clin Exp Dermatol. Mar 2012;37(2):149-52. [Medline].
Ysebaert L, Truc G, Dalac S, et al. Ultimate results of radiation therapy for T1-T2 mycosis fungoides (including reirradiation). Int J Radiat Oncol Biol Phys. Mar 15 2004;58(4):1128-34. [Medline].
Onozuka T, Yokota K, Kawashima T, et al. An elderly patient with mycosis fungoides successfully treated with chronic low-dose oral etoposide therapy. Clin Exp Dermatol. Jan 2004;29(1):91-2. [Medline].
Dummer R, Urosevic M, Kempf W, Kazakov D, Burg G. Imiquimod induces complete clearance of a PUVA-resistant plaque in mycosis fungoides. Dermatology. 2003;207(1):116-8. [Medline].
Apisarnthanarax N, Talpur R, Ward S, Ni X, Kim HW, Duvic M. Tazarotene 0.1% gel for refractory mycosis fungoides lesions: an open-label pilot study. J Am Acad Dermatol. Apr 2004;50(4):600-7. [Medline].
Singh F, Lebwohl MG. Cutaneous T-cell lymphoma treatment using bexarotene and PUVA: a case series. J Am Acad Dermatol. Oct 2004;51(4):570-3. [Medline].
Jones GW, Hoppe RT, Glatstein E. Electron beam treatment for cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):1057-76. [Medline].
Leverkus M, Rose C, Bröcker EB, Goebeler M. Follicular cutaneous T-cell lymphoma: beneficial effect of isotretinoin for persisting cysts and comedones. Br J Dermatol. Jan 2005;152(1):193-4. [Medline].
Jacob R, Scala M, Fung MA. A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy. J Am Acad Dermatol. Jan 2009;60(1):152-4. [Medline].
Russell-Jones R. Extracorporeal photopheresis in cutaneous T-cell lymphoma. Inconsistent data underline the need for randomized studies. Br J Dermatol. Jan 2000;142(1):16-21. [Medline].
Lundin J, Hagberg H, Repp R, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. Jun 1 2003;101(11):4267-72. [Medline].
Fukushima T, Horio K, Matsuo E, et al. Successful cord blood transplantation for mycosis fungoides. Int J Hematol. Dec 2008;88(5):596-8. [Medline].
Tsuji H, Wada T, Murakami M, et al. Two cases of mycosis fungoides treated by reduced-intensity cord blood transplantation. J Dermatol. Dec 2010;37(12):1040-5. [Medline].
Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ, et al. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma (CTCL). Blood. Mar 6 2012;[Medline].
Lambert WC, Cohen PJ, Schwartz RA. Surgical management of mycosis fungoides. J Med. 1997;28(3-4):211-22. [Medline].
Foss F. Overview of cutaneous T-cell lymphoma: prognostic factors and novel therapeutic approaches. Leuk Lymphoma. 2003;44 Suppl 3:S55-61. [Medline].
Kaye FJ, Bunn PA Jr, Steinberg SM, et al. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med. Dec 28 1989;321(26):1784-90. [Medline].
Meyer N, Paul C, Misery L. Pruritus in cutaneous T-cell lymphomas: frequent, often severe and difficult to treat. Acta Derm Venereol. 2010;90(1):12-7. [Medline].
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. Nov 1 2010;28(31):4730-9. [Medline].
Tancrede-Bohin E, Ionescu MA, de La Salmoniere P, et al. Prognostic value of blood eosinophilia in primary cutaneous T-cell lymphomas. Arch Dermatol. Sep 2004;140(9):1057-61. [Medline].
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. Feb 2002;138(2):191-8. [Medline].
Scarisbrick JJ, Whittaker S, Evans AV, et al. Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma. Blood. Feb 1 2001;97(3):624-30. [Medline].
Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). Br J Haematol. Nov 1991;79(3):428-37. [Medline].
Fink-Puches R, Zenahlik P, Back B, Smolle J, Kerl H, Cerroni L. Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients. Blood. Feb 1 2002;99(3):800-5. [Medline].
Bekkenk MW, Jansen PM, Meijer CJ, Willemze R. CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature. Ann Oncol. Jul 2004;15(7):1097-108. [Medline].
Mraz-Gernhard S, Natkunam Y, Hoppe RT, LeBoit P, Kohler S, Kim YH. Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course. J Clin Oncol. Apr 15 2001;19(8):2179-88. [Medline].
Burg G, Kempf W. Therapy of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005:475-528.
Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. Nov 2005;32(10):647-74. [Medline].
| Stage | Skin Lesions | Lymphadenopathy | Erythroderma | Histological Lymphoma | |||
| Patches < 10% | Plaques ³10% | Tumors | Lymph Nodes | Viscera | |||
| IA | + | - | - | - | - | - | - |
| IB | + or - | + | - | - | - | - | - |
| IIA | + or - | + or - | + or - | + | - | - | - |
| IIB | + or - | + or - | + | + or - | - | - | - |
| III | + or - | + or - | + or - | + or - | + | - | - |
| IVA | + or - | + or - | + or - | + or - | + or - | + | - |
| IVB | + or - | + or - | + or - | + or - | + or - | + or - | + |
| Stage Class | Stage | Definition |
| T (Tumor) | T1 | Patches/plaques involving < 10% of body surface |
| T2 | Patches/plaques involving ³10% of body surface | |
| T3 | Tumor(s) present on skin | |
| T4 | Erythroderma | |
| N (Nodes) | N0 | No enlarged lymph node present |
| N1 | Enlarged lymph nodes, histologically uninvolved | |
| N2 | No enlarged lymph node; one or more nodes histologically involved* | |
| N3 | Enlarged lymph nodes, histologically involved | |
| M (Metastasis to viscera) | M0 | No visceral lesion present |
| M1 | Visceral involvement | |
| B (Blood involvement) | B0 | Circulating atypical lymphocytes (Sézary cells) £5% of lymphocytes |
| B1 | Circulating atypical lymphocytes ³5% of lymphocytes (Sézary syndrome) | |
| *Uncommon finding, usually not considered/investigated. | ||
| Clinical Stage | TNM (B) Stage | |||
| IA | T1 N0 M0 | |||
| IIB | T2 N0 M0 | |||
| IIA | T1 N1 M0 | T2 N1 M0 | ||
| IIB | T3 N0 M0 | T3 N1 M0 | ||
| III | T4 N0 M0 | T4 N1 M0 | ||
| IVA | T1 N2 M0 | T2 N2 M0 | T3 N2 M0 | T4 N2 M0 |
| T1 N3 M0 | T2 N3 M0 | T3 N3 M0 | T4 N3 M0 | |
| IVB | T1 N0 M1 | T2 N0 M1 | T3 N0 M1 | T4 N0 M1 |
| T1 N1 M1 | T2 N1 M1 | T3 N1 M1 | T4 N1 M1 | |
| T1 N2 M1 | T2 N2 M1 | T3 N2 M1 | T4 N2 M1 | |
| T1 N3 M1 | T2 N3 M1 | T3 N3 M1 | T4 N3 M1 | |

