Cutaneous Lymphomas

  • Laura Y. McGirt
  • Matthias Steinhoff

Core Messages

  • Cutaneous lymphomas are malignancies of the skin composed most commonly of aberrant lymphocytes from either a B or T-cell lineage. These lymphomas manifest in heterogenous clinical presentations, including the subtle eczematous patch to the more observable ulcerated nodules and tumors. There is also marked variation in morbidity and mortality, ranging from little to no effect on lifespan, to a devas-tatingly rapid progression to death. Numerous therapeutic options have become available in the last few decades, including extracorporeal photopheresis, monoclonal antibodies, and his-tone deacetylase inhibitors, and the choice of treatment is generally based upon the clinical staging and histologic diagnosis. Unfortunately, many of the more aggressive cutaneous lym-phomas will ultimately lead to death despite therapeutic intervention. In this chapter, we will focus on the clinical features, diagnosis, and management of primary cutaneous B and T-cell lymphomas.


Mycosis Fungoides Cutaneous Lymphoma Denileukin Diftitox Lymphomatoid Papulosis Extracorporeal Photopheresis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Groves FD, Linet MS, Travis LB et al (2000) Cancer surveillance series: non-Hodgkin's lymphoma incidence by histologic subtype in the United States from 1978 through 1995. J Natl Cancer Inst 92:1240–1251PubMedCrossRefGoogle Scholar
  2. 2.
    Criscione VD, Weinstock MA (2007) Incidence of cutaneous T-cell lymphoma in the United States, 1973–2002. Arch Dermatol 143:854–859PubMedCrossRefGoogle Scholar
  3. 3.
    Weinstock MA, Horm JW (1988) Mycosis fungoides in the United States. Increasing incidence and descriptive epidemiology. JAMA 260:42–46Google Scholar
  4. 4.
    Vakeva L, Pukkala E, Ranki A (2000) Increased risk of secondary cancers in patients with primary cutaneous T cell lymphoma. J Invest Dermatol 115:62–65PubMedCrossRefGoogle Scholar
  5. 5.
    Huang KP, Weinstock MA, Clarke CA, McMillan A, Hoppe RT, Kim YH (2007) Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: evidence from population-based and clinical cohorts. Arch Dermatol 143(1):45–50PubMedCrossRefGoogle Scholar
  6. 6.
    Burg G, Dummer R, Haeffner A, (2001) From inflammation to neoplasia: mycosis fungoides evolves from reactive inflam-matory conditions (lymphoid infiltrates) transforming into neoplastic plaques and tumors. Arch Dermatol 137:949–952PubMedGoogle Scholar
  7. 7.
    Tan RS, Butterworth CM, McLaughlin H et al (1974) Mycosis fungoides –a disease of antigen persistence. Br J Dermatol 91:607–616PubMedCrossRefGoogle Scholar
  8. 8.
    Dereure O, Levi E, Vonderheid EC, Kadin ME (2002) Infrequent Fas mutations but no Bax or p53 mutations in early mycosis fungoides: a possible mechanism for the accumulation of malignant T lymphocytes in the skin. J Invest Dermatol 118:949–956PubMedCrossRefGoogle Scholar
  9. 9.
    Karenko L, Hahtola S, Paivinen S et al (2005) Primary cutaneous T-cell lymphomas show a deletion or transloca-tion affecting NAV3, the human UNC-53 homologue. Cancer Res 65:8101–8110PubMedCrossRefGoogle Scholar
  10. 10.
    Lessin SR, Vowels BR, Rook AH (1994) Retroviruses and cutaneous T-cell lymphoma. Dermatol Clin 12:243–253PubMedCrossRefGoogle Scholar
  11. 11.
    Mao X, Orchard G, Lillington DM et al (2003) Amplification and overexpression of JUNB is associated with primary cutaneous T-cell lymphomas. Blood 101:1513–1519PubMedCrossRefGoogle Scholar
  12. 12.
    Scarisbrick JJ, Mitchell TJ, Calonje E et al (2003) Microsatellite instability is associated with hypermethyla-tion of the hMLH1 gene and reduced gene expression in mycosis fungoides. J Invest Dermatol 121:894–901PubMedCrossRefGoogle Scholar
  13. 13.
    van Doorn R, Dijkman R, Vermeer MH et al (2002) A novel splice variant of the Fas gene in patients with cutaneous T-cell lymphoma. Cancer Res 62:5389–5392PubMedGoogle Scholar
  14. 14.
    Jackow CM, Cather JC, Hearne V et al (1997) Association of erythrodermic cutaneous T-cell lymphoma, superantigen-positive Staphylococcus aureus, and oligoclonal T-cell receptor V beta gene expansion. Blood 89:32–40PubMedGoogle Scholar
  15. 15.
    Abrams JT, Balin BJ, Vonderheid EC (2001) Association between Sezalry T cell-activating factor, Chlamydia pneu-moniae, and cutaneous T cell lymphoma. Ann N Y Acad Sci 941:69–85PubMedCrossRefGoogle Scholar
  16. 16.
    Knol AC, Quéreux G, Pandolfino MC et al (2005) Presence of Epstein—Barr virus in Langerhans cells of CTCL lesions. J Invest Dermatol 124:280–282PubMedCrossRefGoogle Scholar
  17. 17.
    Herne KL, Talpur R, Breuer-McHam J, et al (2003) Cyto-megalovirus seropositivity is significantly associated with mycosis fungoides and Sézary syndrome. Blood 1012:2132–5CrossRefGoogle Scholar
  18. 18.
    Bazarbachi A, Soriano V, Pawson R et al (1997) Mycosis fun-goides and Sezary syndrome are not associated with HTLV-I infection: an international study. Br J Haematol 98:927–933PubMedCrossRefGoogle Scholar
  19. 19.
    Hinuma Y, Nagata K, Hanaoka M et al (1981) Adult T-cell leukemia: antigen in an ATL cell line and detection of antibodies to the antigen in human sera. Proc Natl Acad Sci U S A 78:6476–6480PubMedCrossRefGoogle Scholar
  20. 20.
    Poiesz BJ, Ruscetti FW, Gazdar AF et al (1980) Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma. Proc Natl Acad Sci U S A 77:7415–7419PubMedCrossRefGoogle Scholar
  21. 21.
    Yoshida M, Miyoshi I, Hinuma Y (1982) Isolation and characterization of retrovirus from cell lines of human adult T-cell leukemia and its implication in the disease. Proc Natl Acad Sci U S A 79:2031–2035PubMedCrossRefGoogle Scholar
  22. 22.
    Berger CL, Hanlon D, Kanada D et al (2002) The growth of cutaneous T-cell lymphoma is stimulated by immature dendritic cells. Blood 99:2929–2939PubMedGoogle Scholar
  23. 23.
    Edelson RL (2001) Cutaneous T cell lymphoma: the helping hand of dendritic cells. Ann N Y Acad Sci 941:1–11PubMedCrossRefGoogle Scholar
  24. 24.
    Nowell PC, Moore Js (1998) Aberrant responses of human lymphocytic neoplasms to cytokine regulation. Immunol Res 17:117CrossRefGoogle Scholar
  25. 25.
    Vowels BR, Lessin SR, Cassin M et al (1994) Th2 cytokine mRNA expression in skin in cutaneous T-cell lymphoma. J Invest Dermatol 103:669–673PubMedCrossRefGoogle Scholar
  26. 26.
    Baykal C et al (2002) Familial mycosis fungoides. Br J Dermatol 146:1108–1110PubMedCrossRefGoogle Scholar
  27. 27.
    Naji AA et al (2001) Mycosis fungoides in identical twins. J Am Acad Dermatol 44:532–533PubMedCrossRefGoogle Scholar
  28. 28.
    Whittaker S (2003) Primary cutaneous T-cell lymphoma. In: Williams H, Bigby M, Diepgen T, Herxheimer A, Naldi L, Rzany B (eds) Evidence-based dermatology. BMJ Books, London, pp 346–372Google Scholar
  29. 29.
    Willemze R, Jaffe ES, Burg G et al (2005) WHOEORTC clas-sification for cutaneous lymphomas. Blood 105:3768–3785PubMedCrossRefGoogle Scholar
  30. 30.
    Zackheim HS, Vonderheid EC, Ramsay DL et al (2000) Relative frequency of various forms of primary cutaneous lymphomas. J Am Acad Dermatol 43:793–796PubMedCrossRefGoogle Scholar
  31. 31.
    Burg G, Kempf W, Haeffner AC et al (1997) Cutaneous lymphomas. Curr Probl Dermatol 9:137–204CrossRefGoogle Scholar
  32. 32.
    Fink-Puches R, Zenahlik P, Bäck B et al (2002) 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 99:800–805PubMedCrossRefGoogle Scholar
  33. 33.
    Pimpinelli N, Santucci M, Carli P et al (1990) Primary cutaneous follicular center cell lymphoma: clinical and histological aspects. Curr Probl Dermatol 19:203–220PubMedGoogle Scholar
  34. 34.
    Condarco T, Sagatys E, Prakash AV et al (2008) Primary cutaneous B-cell lymphoma in a child. Fetal Pediatr Pathol 27:206–214PubMedCrossRefGoogle Scholar
  35. 35.
    Cerroni L, Zochling N, Putz B et al (1997) Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol 24:457–461PubMedCrossRefGoogle Scholar
  36. 36.
    Goodlad JR, Davidson MM, Hollowood K et al (2000) Cutaneous B-cell lymphoma and Borrelia burgdorferi infection in patients from the Highlands of Scotland. Am J Surg Pathol 24:1279–1285PubMedCrossRefGoogle Scholar
  37. 37.
    Dillon WI, Saed GM, Fivenson DP (1995) Borrelia burg-dorferi DNA is undetectable by polymerase chain reaction is skin lesions of morphea, scleroderma, or lichen sclerosis et atrophicus of patients from North America. J Am Acad Dermatol 33:617–620PubMedCrossRefGoogle Scholar
  38. 38.
    LeBoit PE, McNutt NS, Reed JA et al (1994) Primary cutaneous immunocytoma: A B-cell lymphoma that can easily be mistaken for cutaneous lymphoid hyperplasia. Am J Surg Pathol 18:969–978PubMedCrossRefGoogle Scholar
  39. 39.
    Wood GS, Kamath NV, Guitart J et al (2001) Absence of Borrelia burgdorferi DNA in cutaneous B-cell lymphomas from the United States. J Cutan Pathol 28:502–507PubMedCrossRefGoogle Scholar
  40. 40.
    Nagore E, Pérez-Ferriols A, Sánchez-Motilla JM et al (2000) Detection of Epstein—Barr virus and human herpes-virus 7 and 8 genomes in primary cutaneous T- and B-cell lymphomas. Br J Dermatol 143:320–323PubMedCrossRefGoogle Scholar
  41. 41.
    Peris K, Niedermeyer H, Cerroni L et al (1994) Detection of Epstein—Barr virus genome in primary cutaneous T and B cell lymphomas and pseudolymphomas. Arch Dermatol Res 286:364–368PubMedCrossRefGoogle Scholar
  42. 42.
    Slater D (1991) Epstein–Barr virus: an aetiological factor in cutaneous lymphoproliferative disorders? J Pathol 165:1–4PubMedCrossRefGoogle Scholar
  43. 43.
    Zöchling N, Pütz B, Wolf P et al (1998) Human herpesvi-rus 8-specific DNA sequences in primary cutaneous B-cell lymphomas. Arch Dermatol 134:246–247PubMedCrossRefGoogle Scholar
  44. 44.
    Belaud-Rotureau MA et al (2008) Inactivation of p16INK4a/CDKN2A gene may be a diagnostic feature of large B cell lymphoma leg type among cutaneous B cell lymphomas. Virchows Arch 452(6):607–620PubMedCrossRefGoogle Scholar
  45. 45.
    Neri A, Fracchiolla NS, Roscetti E et al (1995) Molecular analysis of cutaneous B- and T-cell lymphomas. Blood 86:3160–3172PubMedGoogle Scholar
  46. 46.
    Senff NJ, Zoutman WH, Vermeer MH, et al (2009) Fine-mapping chromosomal loss at 9p21: correlation with prognosis in primary cutaneous diffuse large B-cell lymphoma, leg type. J Invest Dermatol. 129(5):1149–55PubMedCrossRefGoogle Scholar
  47. 47.
    Weiss LM, Warnke RA, Sklar J et al (1987) Molecular analysis of the t(14;18) chromosomal translocation in malignant lymphomas. N Engl J Med 317:1185–1189PubMedCrossRefGoogle Scholar
  48. 48.
    Florell SR, Cessna M, Lundell RB et al (2006) Usefulness (or lack thereof) of immunophenotyping in atypical cutaneous T-cell infiltrates. Am J Clin Pathol 125:727–736PubMedCrossRefGoogle Scholar
  49. 49.
    Ralfkiaer E (1994) Controversies and discussion on early diagnosis of cutaneous T-cell lymphoma: phenotyping. Dermatol Clin 12:329–334PubMedGoogle Scholar
  50. 50.
    Gerami P, Rosen S, Kuzel T et al (2008) Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 144:738–746PubMedCrossRefGoogle Scholar
  51. 51.
    Benton EC, Morris SL, Robson A et al (2008) An unusual case of granulomatous slack skin disease with necrobiosis. Am J Dermatopathol 30:462–465PubMedCrossRefGoogle Scholar
  52. 52.
    Clarijs M, Poot F, Laka A et al (2003) Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology 206:393–397PubMedCrossRefGoogle Scholar
  53. 53.
    Lutzner M, Edelson R, Schein P et al (1975) Cutaneous T-cell lymphomas: the Sezary syndrome, mycosis fungoi-des, and related disorders. Ann Intern Med 83:534–552PubMedGoogle Scholar
  54. 54.
    Vonderheid EC, Bernengo MG, Burg G et al (2002) Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol 46:95–106PubMedCrossRefGoogle Scholar
  55. 55.
    Felgar RE, Salhany KE, Macon WR et al (1999) The expression of TIA-1+ cytolytic-type granules and other cytolytic lymphocyte-associated markers in CD30+ ana-plastic large cell lymphomas (ALCL): correlation with morphology, immunophenotype, ultrastructure, and clinical features. Hum Pathol. 30(2):228–36PubMedCrossRefGoogle Scholar
  56. 56.
    Bekkenk M, Geelen FAMJ, van Voorst Vader PC et al (2000) Primary and Secondary cutaneous CD30+ lym-phoproliferative disorders: long-term follow up data of 219 patients and guidelines for diagnosis and treatment. A report from the Dutch Cutaneous Lymphoma Group. Blood 95:3653–3661PubMedGoogle Scholar
  57. 57.
    Kempf W, Kutzner H, Cozzio A et al (2008) MUM1 expression in cutaneous CD30+ lymphoproliferative disorders: a valuable tool for the distinction between lympho-matoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Br J Dermatol 158:1280–1287PubMedCrossRefGoogle Scholar
  58. 58.
    Assaf C, Hirsch B, Wagner F et al (2007) Differential expression of TRAF1 aids in the distinction of cutaneous CD30-positive lymphoproliferations. J Invest Dermatol. 127(8):1898–904PubMedCrossRefGoogle Scholar
  59. 59.
    Boulland ML, Wechsler J, Bagot M et al (2000) Primary CD30-positive cutaneous T-cell lymphomas and lympho-matoid papulosis frequently express cytotoxic proteins. Histopathology 36(2):136–44PubMedCrossRefGoogle Scholar
  60. 60.
    Shimoyama M (1991) Diagnostic criteria and clinical subtypes of ATLL. A report from the Lymphoma Study Group (1984–87). Br J Haematol. 79(3):428–37PubMedCrossRefGoogle Scholar
  61. 61.
    Chan JK, Sin VC, Wong KF et al (1997) Nonnasal lym-phoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 89:4501–4513PubMedGoogle Scholar
  62. 62.
    Tai YC, Kim LH, Peh SC (2004) High frequency of EBV association and 30-bp deletion in the LMP-1 gene in CD56 lympho-mas of the upper aerodigestive tract. Pathol Int 54:158–166PubMedCrossRefGoogle Scholar
  63. 63.
    Gaal K, Sun NC, Hernandez AM et al (2000) Sinonasal NK/T-cell lymphomas in the United States. Am J Surg Pathol 24:1511–1517PubMedCrossRefGoogle Scholar
  64. 64.
    Cheung MM, Chan JK, Lau WH et al (1998) Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients. J Clin Oncol 16:70–77PubMedGoogle Scholar
  65. 65.
    Massone C, Chott A, Metze D et al (2004) Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunopheno-typic, and molecular study of 50 patients. Am J Surg Pathol 28:719–735PubMedCrossRefGoogle Scholar
  66. 66.
    Salhany KE, Macon WR, Choi JK et al (1998) Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol 22:881–893PubMedCrossRefGoogle Scholar
  67. 67.
    Toro JR, Liewehr DJ, Pabby N et al (2003) Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood 101:3407–3412PubMedCrossRefGoogle Scholar
  68. 68.
    Cerroni L, Volkenandt M, Rieger E et al (1994) Bcl-2 protein expression and correlation with the interchromosomal 14;18 translocation in cutaneous lymphomas and pseudo-lymphomas. J Invest Dermatol 102:231–235PubMedCrossRefGoogle Scholar
  69. 69.
    Kono T, Nagayasu TS, Nakanishi T et al (2000) Granu-lomatous slack skin: Successful treatment with recombi-nant interferon-gamma. Br J Dermatol 142:353–357PubMedCrossRefGoogle Scholar
  70. 70.
    Cerroni L, Arzberger E, Pütz B et al (2000) Primary cutaneous follicle center cell lymphoma with follicular growth pattern. Blood. 95(12):3922–8PubMedGoogle Scholar
  71. 71.
    de Leval L, Harris NL, Longtine J et al (2001) Cutaneous B-cell lymphomas of follicular and marginal zone types: use of Bcl-6, CD10, Bcl-2, and CD21 in differential diagnosis and classification. Am J Surg Pathol 25:732–741PubMedCrossRefGoogle Scholar
  72. 72.
    Kodama K, Massone C, Chott A et al (2005) Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 106:2491–2497PubMedCrossRefGoogle Scholar
  73. 73.
    Perniciaro C, Winkelmann RK, Daoud MS, (1995) Malignant angioendotheliomatosis is an angiotropic intravascular lym-phoma: immunohistochemical, ultrastructural and molecular genetic studies. Am J Dermatopathol 17:242–248PubMedCrossRefGoogle Scholar
  74. 74.
    Röglin J, Böer A (2007) Skin manifestations of intravascu-lar lymphoma mimic inflammatory diseases of the skin. Br J Dermatol 157:16–25PubMedCrossRefGoogle Scholar
  75. 75.
    Senff NJ, Kluin-Nelemans HC, Willemze R (2008) Results of bone marrow examination in 275 patients with histologi-cal features that suggest an indolent type of cutaneous B-cell lymphoma. Br J Haematol 142:52–56PubMedCrossRefGoogle Scholar
  76. 76.
    Olsen E, Vonderheid E, Pimpinelli N et al (2007) 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. 110(6):1713–22PubMedCrossRefGoogle Scholar
  77. 77.
    Kim YH, Willemze R, Pimpinelli N et al (2007) TNM clas-sification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary 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 110:479–84PubMedCrossRefGoogle Scholar
  78. 78.
    Girardi M, Heald PW, Wilson LD (2004) The pathogenesis of mycosis fungoides. N Engl J Med 350:1978–1988PubMedCrossRefGoogle Scholar
  79. 79.
    Golling P, Cozzio A, Dummer R et al (2008) Primary cutaneous B-cell lymphomas — clinicopathological, prognostic and therapeutic characterisation of 54 cases according to the WHO-EORTC classification and the ISCL/EORTC TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome. Leuk Lymphoma 49:1094–1103PubMedCrossRefGoogle Scholar
  80. 80.
    Siegel RS, Pandolfino T, Guitart J et al (2000) Primary cutaneous Tcell lymphoma: review and current concepts. J Clin Oncol 18:2908–2925PubMedGoogle Scholar
  81. 81.
    Herrmann Jr JJ, Roenigk HH, Hurria A et al (1995) Treatment of mycosis fungoides with photochemotherapy (PUVA): long-term follow-up. J Am Acad Dermatol 33:234–242PubMedCrossRefGoogle Scholar
  82. 82.
    Ramsay DL, Lish KM, Yalowitz CB et al (1992) Ultraviolet-B phototherapy for early-stage cutaneous T-cell lymphoma. Arch Dermatol 128:931–933PubMedCrossRefGoogle Scholar
  83. 83.
    Whittaker SJ, Marsden JR, Spittle M et al (2003) Joint British Association of Dermatologists and UK Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol 149:1095–1107PubMedCrossRefGoogle Scholar
  84. 84.
    Kim YH, Martinez G, Varghese A (2003) Topical nitrogen mustard in the management of mycosis fungoides: update of the Stanford experience. Arch Dermatol 139:165–173PubMedCrossRefGoogle Scholar
  85. 85.
    Trautinger F, Knobler R, Willemze R et al (2006) EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer 42:1014–1030PubMedCrossRefGoogle Scholar
  86. 86.
    Jumbou O, N'Guyen JM, Tessier MH et al (1999) Long-term follow-up in 51 patients with mycosis fungoides and Sezary syndrome treated by interferon-alfa. Br J Dermatol 140:427–431PubMedCrossRefGoogle Scholar
  87. 87.
    Jones GW, Hoppe RT, Glatstein E (1995) Electron beam treatment for cutaneous T-cell lymphoma. Hematol Oncol Clin North Am 9:1057–1076PubMedGoogle Scholar
  88. 88.
    Olsen EA, Kim YH, Kuzel TM et al (2007) Phase IIb mul-ticenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma. J Clin Oncol 25:3109–3115PubMedCrossRefGoogle Scholar
  89. 89.
    Zackheim HS, Kashani-Sabet M, Hwang ST (1996) Low-dose methotrexate to treat erythrodermic cutaneous T-cell lymphoma: results in twenty-nine patients. J Am Acad Dermatol 34:626–631PubMedCrossRefGoogle Scholar
  90. 90.
    Knobler R, Jantschitsch C (2003) Extracorporeal photo-chemoimmunotherapy in cutaneous T-cell lymphoma. Transfus Apheresis Sci 28:81–89CrossRefGoogle Scholar
  91. 91.
    Kim YH, Jensen RA, Watanabe GL et al (1996) Clinical stage IA (limited patch and plaque) mycosis fungoides. A long-term outcome analysis. Arch Dermatol 132:1309–1313CrossRefGoogle Scholar
  92. 92.
    Zackheim HS, Amin S, Kashani-Sabet M et al (1999) Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients. J Am Acad Dermatol 40:418–425PubMedCrossRefGoogle Scholar
  93. 93.
    Bonta MD, Tannous ZS, Demierre MF et al (2000) Rapidly progressing mycosis fungoides presenting as follicular mucinosis. J Am Acad Dermatol 43:635–640PubMedCrossRefGoogle Scholar
  94. 94.
    van Doorn R, Scheffer E, Willemze R (2001) Follicular mycosis fungoides: a distinct disease entity with or without associated follicular mucinosis. Arch Dermatol 138:191–198CrossRefGoogle Scholar
  95. 95.
    Balus L, Manente L, Remotti D et al (1996) Granulomatous slack skin. Report of a case and review of the literature. Am J Dermatopathol 18:199–206CrossRefGoogle Scholar
  96. 96.
    Wollina U, Graefe T, Füller J (2002) Granulomatous slack skin or granulomatous mycosis fungoides: a case report. Complete response to percutaneous radiation and inter-feron alpha. J Cancer Res Clin Oncol 128:50–54Google Scholar
  97. 97.
    Kanthilatha P, Ravikala R, Rama D et al (2000) Granu-lomatous slack skin. Int J Dermatol 39:363–382CrossRefGoogle Scholar
  98. 98.
    Van Haselen CW, Toonstra J, Van der Putte SJC et al (1998) Granulomatous slack skin: Report of three patients with an updated review of the literature. Dermatology 196:382–391CrossRefGoogle Scholar
  99. 99.
    Gottlieb SL, Wolfe JT, Fox FE et al (1996) Treatment of cutaneous T-cell lymphoma with extracorporeal photo-pheresis monotherapy and in combination with recombi-nant interferon alfa: a 10-year experience at a single institution. J Am Acad Dermatol 35:946–957PubMedCrossRefGoogle Scholar
  100. 100.
    Edelson R, Berger C, Gasparro F et al (1987) Treatment of cutaneous T-cell lymphoma by extracorporeal photochemo-therapy. Preliminary results. N Engl J Med 316:297–303PubMedCrossRefGoogle Scholar
  101. 101.
    Evans AV, Wood BP, Scarisbrick JJ et al (2001) Extracorporeal photopheresis in Sezary syndrome: hema-tologic parameters as predictors of response. Blood 98:1298–1301PubMedCrossRefGoogle Scholar
  102. 102.
    Suchin KR, Cucchiara AJ, Gottleib SL et al (2002) Treatment of cutaneous T-cell lymphoma with combined immunomodulatory therapy: a 14-year experience at a single institution. Arch Dermatol 138:1054–1060PubMedCrossRefGoogle Scholar
  103. 103.
    Bunn Jr PA, Ihde DC, Foon KA (1986) The role of recom-binant interferon alfa-2a in the therapy of cutaneous T-cell lymphomas. Cancer 57:1689–1695PubMedCrossRefGoogle Scholar
  104. 104.
    Olsen EA, Rosen ST, Vollmer RT et al (1989) Interferon alfa-2a in the treatment of cutaneous T cell lymphoma. J Am Acad Dermatol 20:395–407PubMedCrossRefGoogle Scholar
  105. 105.
    Olsen E, Duvic M, Frankel A et al (2001) Pivotal phase III trial of two dose levels of denileukin diftitox for the treatment of cutaneous T-cell lymphoma. J Clin Oncol 19:376–388PubMedGoogle Scholar
  106. 106.
    Winkelmann RK, Diaz-Perez JL, Buechner SA (1984) The treatment of Sezary syndrome. J Am Acad Dermatol 10:1000–1004PubMedCrossRefGoogle Scholar
  107. 107.
    Duvic M, Hymes K, Heald P et al (2001) Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II–III trial results. J Clin Oncol 19:2456–2471PubMedGoogle Scholar
  108. 108.
    Zackheim HS, Epstein Jr EH (1989) Low-dose methotrexate for the Sezary syndrome. J Am Acad Dermatol 21:757–762PubMedCrossRefGoogle Scholar
  109. 109.
    Lundin J, Hagberg H, Repp R et al (2003) Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood 101:4267–4272PubMedCrossRefGoogle Scholar
  110. 110.
    Bernengo MG, Quaglino P, Comessatti A et al (2007) Low-dose intermittent alemtuzumab in the treatment of Sézary syndrome: clinical and immunologic findings in 14 patients. Haematologica 92(6):784–94PubMedCrossRefGoogle Scholar
  111. 111.
    Duvic M, Talpur R, Wen S et al (2006) Phase II evaluation of gemcitabine monotherapy for cutaneous T-cell lym-phoma Clin Lymphoma Myeloma 7(1):51–8CrossRefGoogle Scholar
  112. 112.
    Akpek G, Koh HK, Bogen S et al (1999) Chemotherapy with etoposide, vincristine, doxorubicin, bolus cyclophos-phamide, and oral prednisone in patients with refractory cutaneous T-cell lymphoma. Cancer 86:1368–1376PubMedCrossRefGoogle Scholar
  113. 113.
    Diamandidou E, Cohen PR, Kurzrock R (1996) Mycosis fungoides and Sezary syndrome. Blood 88:2385–2409PubMedGoogle Scholar
  114. 114.
    Liu HL, Hoppe RT, Kohler S et al (2003) Cd30+ cutaneous lymphoproliferative disorders: the stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol 49:1049–1058PubMedCrossRefGoogle Scholar
  115. 115.
    Yu JB, McNiff JM, Lund MW, Wilson LD (2008) Treatment of primary cutaneous CD30+ anaplastic large-cell lym-phoma with radiation therapy. Int J Radiat Oncol Biol Phys 70:1542–1545PubMedCrossRefGoogle Scholar
  116. 116.
    Vonderheid EC, Sajjadian A, Kadin ME (1996) Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. J Am Acad Dermatol 34:470–481PubMedCrossRefGoogle Scholar
  117. 117.
    Beljaards RC, Willemze R (1992) The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol 126:596–602PubMedCrossRefGoogle Scholar
  118. 118.
    Ishitsuka K, Tamura K (2008) Treatment of adult T-cell leukemia/lymphoma: past, present, and future. Eur J Haematol 80:185–196PubMedCrossRefGoogle Scholar
  119. 119.
    Shimizu S, Yasui C, Koizumi K et al (2007) Cutaneous-type adult T-cell leukemia/lymphoma presenting as a solitary large skin nodule: a review of the literature. J Am Acad Dermatol 57:S115–S117PubMedCrossRefGoogle Scholar
  120. 120.
    Lee J, Park YH, Kim WS et al (2005) Extranodal nasal type NK/T-cell lymphoma: elucidating clinical prognostic factors for risk-based stratification of therapy. Eur J Cancer 41:1402–1408PubMedCrossRefGoogle Scholar
  121. 121.
    Yamada Y, Tomonaga M (2003) The current status of therapy for adult T-cell leukaemia-lymphoma in Japan. Leuk Lymphoma 44:611–618PubMedCrossRefGoogle Scholar
  122. 122.
    Shimoyama M (1992) Treatment of patients with adult T-cell leukemia-lymphoma: an overview. In: Takatsuki K, Hinuma Y, Yoshida M (eds) Advances in adult T-cell leukemia and HTLV-I research. Japan Scientific Societies Press, Tokyo, pp43–56Google Scholar
  123. 123.
    Rojnuckarin P, Nakorn TN, Assanasen T et al (2007) Cyclosporin in subcutaneous panniculitis-like T-cell lym-phoma. Leuk Lymphoma 48:560–563PubMedCrossRefGoogle Scholar
  124. 124.
    Willemze R, Jansen PM, Cerroni L et al (2008) Subcutaneous panniculitis-like T-cell lymphoma: defini-tion, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 111:838–845PubMedCrossRefGoogle Scholar
  125. 125.
    Bekkenk MW, Vermeer MH, Jansen PM et al (2003) Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood 102:2213–2219PubMedCrossRefGoogle Scholar
  126. 126.
    Heinzerling LM, Urbanek M, Funk JO et al (2000) Reduction of tumor burden and stabilization of disease by systemic therapy with anti-CD20 antibody (rituximab) in patients with primarycutaneous B-cell lymphoma. Cancer 89:1835–1844PubMedCrossRefGoogle Scholar
  127. 127.
    Roguedas AM, Watier H, Paintaud G et al (2005) Intralesional therapy with anti-CD20 monoclonal antibody rituximab: local and systemic efficacy in primary cutaneous B-cell lymphoma. Br J Dermatol. 152(3):541–4PubMedCrossRefGoogle Scholar
  128. 128.
    Bunn Jr PA, Lamberg SI (1979) Report of the committee on staging and classification of cutaneous T-cell lympho-mas. Cancer Treat Rep 63:725–728PubMedGoogle Scholar
  129. 129.
    Zenahlik P, Pink-Fuches R, Kapp KS et al (2000) Therapy of primary cutaneous B-cell lymphomas. Hautarzt 51:19–24PubMedCrossRefGoogle Scholar
  130. 130.
    Morales AV, Advani R, Horwitz SM et al (2008) Indolent primary cutaneous B-cell lymphoma: experience using systemic rituximab. J Am Acad Dermatol 59:953–957PubMedCrossRefGoogle Scholar
  131. 131.
    Li C, Inagaki H, Kuo TT et al (2003) Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopatho-logic study of 24 Asian cases. Am J Surg Pathol 27:1061–1069PubMedCrossRefGoogle Scholar
  132. 132.
    Vermeer MH, Geelen FAMJ, van Haselen CW et al (1996) Primary cutaneous large B-cell lymphomas of the legs: a distinct type of cutaneous B-cell lymphoma with an intermediate prognosis. Arch Dermatol 132:1304–1308PubMedCrossRefGoogle Scholar
  133. 133.
    Bekkenk M, Vermeer MH, Geerts ML et al (1999) Treatment of multifocal primary cutaneous B-cell lym-phoma: guidelines of the Dutch Cutaneous Lymphoma Group. J Clin Oncol 17:2471–2478PubMedGoogle Scholar
  134. 134.
    Maza S, Gellrich S, Assaf C et al (2008) Yttrium-90 ibritu-momab tiuxetan radioimmunotherapy in primary cutaneous B-cell lymphomas: first results of a prospective, monocentre study. Leuk Lymphoma 49(9):1702–9PubMedCrossRefGoogle Scholar
  135. 135.
    Grange F, Bekkenk MW, Wechsler J et al (2001) Prognostic factors in primary cutaneous large B-cell lymphomas: A European Multicenter Study. J Clin Oncol 19:3602–3610PubMedGoogle Scholar
  136. 136.
    Shimada K, Matsue K, Yamamoto K et al (2008) Retrospective analysis of intravascular large B-cell lym-phoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 26:3189–3195PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2010

Authors and Affiliations

  • Laura Y. McGirt
    • 1
  • Matthias Steinhoff
  1. 1.Department of DermatologyJohns Hopkins School of MedicineBaltimoreUSA

Personalised recommendations