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Lymphomatoid Papulosis

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Atlas of Cutaneous Lymphomas

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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.

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References

  1. Macaulay WL. Lymphomatoid papulosis: a continuing self healing eruption, clinically benign — histologically malignant. Arch Dermatol. 1968;97:23–30.

    Article  CAS  PubMed  Google Scholar 

  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. 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.

    CAS  PubMed  Google Scholar 

  4. Duvic M. CD30+ neoplasms of the skin. Curr Hematol Malig Rep. 2011;6:245–50.

    Article  PubMed  Google Scholar 

  5. Kadin ME. Lymphomatoid papulosis and associated lymphomas. How are they related? Arch Dermatol. 1993;129:351–3.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  7. Beljaards RC, Willemze R. The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol. 1992;126:596–602.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  9. LeBoit PE. Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. 1996;18:221–35.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  18. Krathen RA, Ward S, Duvic M. Bexarotene is a new treatment option for lymphomatoid papulosis. Dermatology. 2003;206:142–7.

    Article  CAS  PubMed  Google Scholar 

  19. Hughes PSH. Treatment of lymphomatoid papulosis with imiquimod 5 % cream. J Am Acad Dermatol. 2006;54:546–7.

    Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  31. Demierre MF, Goldberg LJ, Kadin ME, Koh HK. Is it lymphoma or lymphomatoid papulosis? J Am Acad Dermatol. 1997;36:765–72.

    Article  CAS  PubMed  Google Scholar 

  32. Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol. 2006;33 Suppl 1:58–70.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, Boston, MA, USA

    Amrita Goyal M.D.

  2. Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joi B. Carter M.D.

  3. Dermatopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Lyn McDivitt Duncan M.D.

Authors
  1. Amrita Goyal M.D.
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  2. Joi B. Carter M.D.
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  3. Lyn McDivitt Duncan M.D.
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Corresponding author

Correspondence to Lyn McDivitt Duncan M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA

    Joi B. Carter

  2. Division of Health Sciences and Technology, Harvard Medical School, Boston, Massachusetts, USA

    Amrita Goyal

  3. Dermatopathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA

    Lyn McDivitt Duncan

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© 2015 Springer International Publishing Switzerland

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Goyal, A., Carter, J.B., Duncan, L.M. (2015). Lymphomatoid Papulosis. 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_11

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  • DOI: https://doi.org/10.1007/978-3-319-17217-0_11

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17216-3

  • Online ISBN: 978-3-319-17217-0

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