Abstract
The skin may be affected by lymphoproliferative diseases either primarily or secondarily. In addition to skin involvement of systemic lymphomas, including Hodgkin disease and non-Hodgkin lymphomas, a heterogeneous group of primary cutaneous lymphomas (extranodal non-Hodgkin lymphomas) originating from B cells, T cells and NK (natural killer) cells, have been described. By definition, these lymphomas are restricted to the skin at the time of diagnosis but may show involvement of lymph nodes, visceral organs, and bone marrow during their course. However, some types of cutaneous lymphomas very rarely involve extracutaneous sites. In the last two decades, many classification systems of these diseases have been proposed. This chapter is mainly organized according to the WHO-EORTC (World Health Organization-European Organization for Research and Treatment of Cancer) classification of cutaneous lymphomas published in 2005 and the WHO classification of lymphomas published in 2008. Most of these primary cutaneous lymphomas included in these classification systems showing different clinical, histopathologic, cytogenetic, and molecular genetic features and prognosis will be discussed. Pseudolymphomas which are clinical and histopathologic imitators of lymphomas, and cutaneous infiltration of hematologic neoplasms are included in this chapter for convenience.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Suggested Reading
Akaraphanth R, Douglass MC, Lim HW. Hypopigmented mycosis fungoides: treatment and a 6(1/2)-year follow-up of 9 patients. J Am Acad Dermatol. 2000;42:33–9.
Baykal C, Buyukbabani N, Kaymaz R. Familial mycosis fungoides. Br J Dermatol. 2002;146:1108–10.
Bekkenk MW, Geelen FA, van Voorst Vader PC, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. 2000;95:3653–61.
Burgdorf WHC, Plewig G, Wolff HH, Landthaler M. Braun-Falco’s dermatology, edn 3. Italy: Springer-Verlag; 2009.
Cerroni L, Gatter K, Kerl H. Skin lymphoma. The illustrated guide, edn 3. Singapore: Wiley-Blackwell; 2009.
Cheeley J, Sahn RE, DeLong LK, Parker SR. Acitretin for the treatment of cutaneous T-cell lymphoma. J Am Acad Dermatol. 2013;68:247–54.
de-Misra RF, Garcia M, Dorta S. Solitary oral ulceration as the first appearance of lymphomatoid papulosis: a diagnostic challange. Clin Exp Dermatol. 2010;35:165–8.
Desai A, Telang GH, Olszewski AJ. Remission of primary cutaneous anaplastic large cell lymphoma after a brief course of brentuximab vedotin. Ann Hematol. 2013;92:567–8.
de Souza A, el-Azhary RA, Camilleri MJ, et al. In search of prognostic indicators for lymphomatoid papulosis: a retrospective study of 123 patients. J Am Acad Dermatol. 2012;66:928–37.
El Shabrawi-Caelen L, Kerl H, Cerroni L. Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C. Arch Dermatol. 2004;140:441–7.
Elcin G, Duman N, Karahan S, et al. Long-term follow-up of early mycosis fungoides patients treated with narrowband ultraviolet B phototherapy. J Dermatolog Treat. 2014;25:268–73.
Fink-Puches R, Chott A, Ardigó M, et al. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol. 2004;21:525–33.
Hanna S, Walsh N, D’Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol. 2006;23:350–4.
Herrmann JL, Hughey LC. Recognizing large-cell transformation of mycosis fungoides. J Am Acad Dermatol. 2012;67:665–72.
Hsu YJ, Su LH, Hsu YL, et al. Localized lymphomatoid papulosis. J Am Acad Dermatol. 2010;62:353–6.
Jain S, Zain J, O’Connor O. Novel therapeutic agents for cutaneous T-Cell lymphoma. J Hematol Oncol. 2012;5:24.
James WD, Berger T, Elston D. Andrew’s diseases of the skin: Clinical Dermatology, edn 11. China: Saunders Elsevier; 2011.
Kazakov DV, Burg G, Kempf W. Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol. 2004;18:397–415.
Kempf W, Kazakov DV, Schärer L, et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol. 2013;37:1–13.
Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood. 2011;118:4024–35.
Kempf W, Rozati S, Kerl K, et al. Cutaneous peripheral T-cell lymphomas, unspecified/NOS and rare subtypes: a heterogeneous group of challenging cutaneous lymphomas. G Ital Dermatol Venereol. 2012;147:553–62.
Knobler R, Berlin G, Calzavara-Pinton P, et al. Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol. 2014;28 (Suppl 1):1–37.
Kunishige JH, McDonald H, Alvarez G, et al. Lymphomatoid papulosis and associated lymphomas: a retrospective case series of 84 patients. Clin Exp Dermatol. 2009;34:576–81.
LeBoit PE, Burg G, Weedon D, Sarasin A. Pathology and genetics of skin tumours. World Health Organization Classification of Tumours. France; IARC Press; 2006.
Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol. 2003;49:1049–58.
Martorell-Calatayud A, Hernandes-Martin A, Colmenero I, et al. Lymphomatoid papulosis in children: report of 9 cases and review of the literature. Actas Dermosifiliorg. 2010;101:693–701.
Nanda A, AlSaleh QA, Al-Ajmi H, et al. Mycosis fungoides in Arab children and adolescents. A report of 36 patients from Kuwait. Pediatr Dermatol. 2010;27:607–13.
Nofal A, Abdel-Mawla MY, Assaf M, Salah E. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation. J Am Acad Dermatol. 2012;67:748–59.
Olsen E, Vonderheid E, Pimpinelli N, et al. ISCL/EORTC. 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; 110:1713–22.
Ozguroglu E, Buyukbabani N, Ozguroglu M, Baykal C. Generalized telangiectasia as the major manifestation of angiotropic (intravascular) lymphoma. Br J Dermatol. 1997;137:422–5.
Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sézary syndrome. Blood. 2009;114:4337–53.
Puddu P, Ferranti G, Frezzolini A, et al. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. J Am Acad Dermatol. 1999;40:298–9.
Rigel DS, Robinson JK, Ross M, Friedman RJ, Corkerell CJ, Lim HW, Stockfleth HW, Kirkwood JM. Cancer of the skin. China: Saunders Elsevier; 2011.
Sokol L, Naghashpour M, Glass LF. Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management. Cancer Control. 2013;19:326–44.
Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, edn 4. Lyon: IARC Press; 2008.
Trautinger F, Knobler R, Willemze R, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer 2006;42:1014–30.
Vonderheid EC, Bernengo MG, Burg G, et al; ISCL. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol. 2002;46:95–106.
Wahie S, Dayala S, Husain A, et al. Cutaneous features of intravascular lymphoma. Clin Exp Dermatol. 2011;36:288–91.
Whittaker SJ, Foss FM. Efficacy and tolerability of currently available therapies for the mycosis fungoides and Sezary syndrome variants of cutaneous T-cell lymphoma. Cancer Treat Rev. 2007;33:146–60.
Wilcox RA. Cutaneous B-cell lymphomas: update on diagnosis, risk-stratification, and management. Am J Hematol. 2013;88:73–6.
Willemze R, Jaffe E, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–85.
Willemze R. Primary cutaneous B-cell lymphoma: classification and treatment. Curr Opin Oncol. 2006;18:425–31.
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.
Zackheim HS, Jones C, Leboit PE, et al. Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses of clonality. J Am Acad Dermatol. 2003;49:620–3.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Baykal, C., Yazganoğlu, K.D. (2014). Cutaneous Lymphomas. In: Clinical Atlas of Skin Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-40938-7_14
Download citation
DOI: https://doi.org/10.1007/978-3-642-40938-7_14
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-40937-0
Online ISBN: 978-3-642-40938-7
eBook Packages: MedicineMedicine (R0)