Advertisement

Lymphomatoid Papulosis

  • Amrita Goyal
  • Joi B. Carter
  • Lyn McDivitt DuncanEmail author

Abstract

Lymphomatoid papulosis (LyP) is a benign CD30+ lymphoproliferative disease that presents as recurrent, spontaneously healing crops of papules and nodules. There are four histologic variants of this lymphoma: types A, B, C, and D. Types B, C, and D resemble more aggressive lymphomas and can be difficult to differentiate from mycosis fungoides (MF), anaplastic large-cell lymphoma (ALCL), and CD8+ aggressive epidermotropic T-cell lymphoma (CD8+ AECTCL). Although LyP is benign and requires minimal treatment, it is occasionally associated with the development of malignant lymphomas, including MF, primary cutaneous ALCL, and Hodgkin lymphoma, thus mandating careful surveillance of patients with LyP. This chapter addresses the clinical presentation, prognosis, and treatment of LyP, followed by a discussion of the immunohistochemical, histopathologic, and molecular characteristics of the four subtypes. It closes with a clinical case.

Keywords

Lymphomatoid papulosis CD30+ lymphoproliferative diseases Bexarotene Ultraviolet phototherapy Anaplastic large cell lymphoma Mycosis fungoides CD8+ aggressive epidermotropic T-cell lymphoma Pityriasis lichenoides et varioliformis acuta Type A Type B Type C Type D 

References

  1. 1.
    Macaulay WL. Lymphomatoid papulosis: a continuing self healing eruption, clinically benign — histologically malignant. Arch Dermatol. 1968;97:23–30.PubMedCrossRefGoogle Scholar
  2. 2.
    Ralfkiaer E, Willemze R, Paulli M, Kadin ME. Primary cutaneous CD30-positive T-cell lymphoproliferative disorders. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumors of hematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer; 2008. p. 300–1.Google Scholar
  3. 3.
    Bekkenk MW, Geelen AMJ, Vader PCVV, Heule F, Geerts M, Van Vloten WA, 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.PubMedGoogle Scholar
  4. 4.
    Duvic M. CD30+ neoplasms of the skin. Curr Hematol Malig Rep. 2011;6:245–50.PubMedCrossRefGoogle Scholar
  5. 5.
    Kadin ME. Lymphomatoid papulosis and associated lymphomas. How are they related? Arch Dermatol. 1993;129:351–3.PubMedCrossRefGoogle Scholar
  6. 6.
    Zackheim HS, Jones C, Leboit PE, Kashani-Sabet M, McCalmont TH, Zehnder J. Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality. J Am Acad Dermatol. 2003;49:620–3.PubMedCrossRefGoogle Scholar
  7. 7.
    Beljaards RC, Willemze R. The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol. 1992;126:596–602.PubMedCrossRefGoogle Scholar
  8. 8.
    De Souza A, El-Azhary RA, Camilleri MJ, Wada DA, Appert DL, Gibson LE. In search of prognostic indicators for lymphomatoid papulosis: a retrospective study of 123 patients. J Am Acad Dermatol. 2012;66:928–37.PubMedCrossRefGoogle Scholar
  9. 9.
    LeBoit PE. Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. 1996;18:221–35.PubMedCrossRefGoogle Scholar
  10. 10.
    McCarty MJ, Vukelja SJ, Sausville EA, Perry JJ, James WD, Jaffe ES, et al. Lymphomatoid papulosis associated with Ki-1-positive anaplastic large cell lymphoma. A report of two cases and a review of the literature. Cancer. 1994;74:3051–8.PubMedCrossRefGoogle Scholar
  11. 11.
    Zackheim HS, LeBoit PE, Gordon BI, Glassberg AB. Lymphomatoid papulosis followed by Hodgkin’s lymphoma. Differential response to therapy. Arch Dermatol. 1993;129:86–91.PubMedCrossRefGoogle Scholar
  12. 12.
    Davis TH, Morton CC, Miller-Cassman R, Balk SP, Kadin ME. Hodgkin’s disease, lymphomatoid papulosis, and cutaneous T-cell lymphoma derived from a common T-cell clone. N Engl J Med. 1992;326:1115–22.PubMedCrossRefGoogle Scholar
  13. 13.
    Wang HH, Myers T, Lach LJ, Hsieh CC, Kadin ME. Increased risk of lymphoid and nonlymphoid malignancies in patients with lymphomatoid papulosis. Cancer. 1999;86:1240–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Cabanillas F, Armitage J, Pugh WC, Weisenburger D, Duvic M. Lymphomatoid papulosis: a T-cell dyscrasia with a propensity to transform into malignant lymphoma. Ann Intern Med. 1995;122:210–7.PubMedCrossRefGoogle Scholar
  15. 15.
    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.PubMedCrossRefGoogle Scholar
  16. 16.
    Eberle FC, Song JY, Xi L, Raffeld M, Harris NL, Wilson WH, et al. Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma. Am J Surg Pathol. 2012;36:716–25.PubMedCentralPubMedCrossRefGoogle Scholar
  17. 17.
    Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, 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.PubMedCentralPubMedCrossRefGoogle Scholar
  18. 18.
    Krathen RA, Ward S, Duvic M. Bexarotene is a new treatment option for lymphomatoid papulosis. Dermatology. 2003;206:142–7.PubMedCrossRefGoogle Scholar
  19. 19.
    Hughes PSH. Treatment of lymphomatoid papulosis with imiquimod 5 % cream. J Am Acad Dermatol. 2006;54:546–7.PubMedCrossRefGoogle Scholar
  20. 20.
    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.PubMedGoogle Scholar
  21. 21.
    Plaza JA, Feldman AL, Magro C. Cutaneous CD30-positive lymphoproliferative disorders with CD8 expression: a clinicopathologic study of 21 cases. J Cutan Pathol. 2013;40:236–47.PubMedCrossRefGoogle Scholar
  22. 22.
    Bertolotti A, Pham-Ledard AL, Vergier B, Parrens M, Bedane C, Beylot-Barry M. Lymphomatoid papulosis type D: an aggressive histology for an indolent disease. Br J Dermatol. 2013;169:1157–9.PubMedCrossRefGoogle Scholar
  23. 23.
    McQuitty E, Curry JL, Tetzlaff MT, Prieto VG, Duvic M, Torres-Cabala C. The differential diagnosis of CD8-positive (“type D”) lymphomatoid papulosis. J Cutan Pathol. 2014;41:88–100.PubMedCrossRefGoogle Scholar
  24. 24.
    Magro CM, Morrison C, Merati K, Porcu P, Wright ED, Crowson AN. CD8+ lymphomatoid papulosis and its differential diagnosis. Am J Clin Pathol. 2006;125:490–501.PubMedCrossRefGoogle Scholar
  25. 25.
    Saggini A, Gulia A, Argenyi Z, Fink-Puches R, Lissia A, Magaña M, et al. A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. Description of 9 cases. Am J Surg Pathol. 2010;34:1168–75.PubMedCrossRefGoogle Scholar
  26. 26.
    Steinhoff M, Hummel M, Anagnostopoulos I, Kaudewitz P, Seitz V, Assaf C, et al. Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin. Blood. 2002;100:578–84.PubMedCrossRefGoogle Scholar
  27. 27.
    Chott A, Vonderheid EC, Olbricht S, Miao NN, Balk SP, Kadin ME. The dominant T cell clone is present in multiple regressing skin lesions and associated T cell lymphomas of patients with lymphomatoid papulosis. J Invest Dermatol. 1996;106:696–700.PubMedCrossRefGoogle Scholar
  28. 28.
    DeCoteau JF, Butmarc JR, Kinney MC, Kadin ME. The t(2;5) chromosomal translocation is not a common feature of primary cutaneous CD30+ lymphoproliferative disorders: comparison with anaplastic large-cell lymphoma of nodal origin. Blood. 1996;87:3437–41.PubMedGoogle Scholar
  29. 29.
    Karai LJ, Kadin ME, Hsi ED, Sluzevich JC, Ketterling RP, Knudson RA, et al. Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis. Am J Surg Pathol. 2013;37:1173–81.PubMedCrossRefGoogle Scholar
  30. 30.
    Paulli M, Berti E, Rosso R, Boveri E, Kindl S, Klersy C, et al. CD30/Ki-1-positive lymphoproliferative disorders of the skin—clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group. J Clin Oncol. 1995;13:1343–54.PubMedGoogle Scholar
  31. 31.
    Demierre MF, Goldberg LJ, Kadin ME, Koh HK. Is it lymphoma or lymphomatoid papulosis? J Am Acad Dermatol. 1997;36:765–72.PubMedCrossRefGoogle Scholar
  32. 32.
    Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol. 2006;33 Suppl 1:58–70.PubMedCrossRefGoogle Scholar
  33. 33.
    Wada DA, Law ME, Hsi ED, Dicaudo DJ, Ma L, Lim MS, et al. Specificity of IRF4 translocations for primary cutaneous anaplastic large cell lymphoma: a multicenter study of 204 skin biopsies. Mod Pathol. 2011;24:596–605.PubMedCentralPubMedCrossRefGoogle Scholar
  34. 34.
    Liu V, Cutler C, Young AZ. Case 38–2007: a 44-year-old woman with generalized, painful, ulcerated skin lesions. N Engl J Med. 2007;357:2496–505.PubMedCrossRefGoogle Scholar
  35. 35.
    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.PubMedCrossRefGoogle Scholar
  36. 36.
    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.PubMedCrossRefGoogle Scholar
  37. 37.
    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.PubMedCrossRefGoogle Scholar
  38. 38.
    Dereure O, Levi E, Kadin ME. T-cell clonality in pityriasis lichenoides et varioliformis acuta: a heteroduplex analysis of 20 cases. Arch Dermatol. 2000;136:1483–6.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
  • Lyn McDivitt Duncan
    • 3
    Email author
  1. 1.Harvard-MIT Division of Health Sciences and TechnologyHarvard Medical SchoolBostonUSA
  2. 2.Department of DermatologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

Personalised recommendations