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Lymphomatoid Papulosis Follow-up

  • Author: John A Zic, MD; Chief Editor: William D James, MD  more...
 
Updated: Dec 04, 2015
 

Further Outpatient Care

Patients with lymphomatoid papulosis (LyP) require long-term follow-up, preferably twice yearly, to monitor the disease and evaluate for the development of associated lymphoma.

Patients on systemic therapy or PUVA phototherapy require closer monitoring for adverse effects.

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Prognosis

The prognosis of lymphomatoid papulosis (LyP) is good because most patients have a chronic, indolent course. A retrospective cohort analysis found that no patients with lymphomatoid papulosis died of the disease, and the overall survival rate was 92% at 5 and 10 years.

Physicians are guardedly optimistic about the prognosis because estimates indicate that as many as 4-25% of patients have a history of associated malignant lymphoma (ALCL, HD, MF) prior to, concurrent with, or subsequent to the diagnosis of lymphomatoid papulosis.[21, 38] Unfortunately, no clinical or histologic factors analyzed to date are predictive of worse outcomes in persons with lymphomatoid papulosis. A study suggested that fascin expression is increased in lymphomatoid papulosis cases associated with a malignant lymphoma.[39] Alterations in transforming growth factor-beta signaling are hypothesized to play a role in the progression of lymphomatoid papulosis to malignant lymphoma. Additionally, data have shown an increased risk of associated lymphomas in lymphomatoid papulosis cases with CCR3+ atypical cells or the 30M362 allelic form of the CD30 promoter.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is more likely than mycosis fungoides (MF) to manifest as an ulcerated tumor and palpable lymph nodes. MF is the most common variant of cutaneous T-cell lymphoma and is characterized by the development of red patches or plaques in sun-protected areas. MF is more likely to manifest as patches and plaques than tumors. Disease-specific survival at 5 and 10 years for pcALCL was 85% in a recent study.

Associated lymphomas more rarely include immunoblastic lymphoma, lethal midline granuloma (currently considered as natural killer cell lymphoma in many patients), and systemic lymphocytic lymphoma. In most patients, the malignancy develops many years after the diagnosis of lymphomatoid papulosis.

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Patient Education

Instruct patients that they are not an infectious risk to others.

Instruct patients about local wound care of open crusted lesions. Lesions should be cleaned with mild soap and water twice daily, and topical petroleum ointment should be applied to prevent infection and aid healing. Crusted lesions should be covered with a loose adhesive bandages until healed.

Instruct patients to contact their physician if any of the following symptoms or signs develop that may herald the onset of infection or associated malignancy:

  • Fever, chills, night sweats, or weight loss
  • Persistent skin nodules or patches that do not regress over a 2- to 3-month period
  • Enlarged lymph node glands in the neck, groin, or axillae
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Contributor Information and Disclosures
Author

John A Zic, MD Associate Professor of Medicine/Dermatology, Director, VU Cutaneous Lymphoma Clinic, Vanderbilt University School of Medicine

John A Zic, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Daniel S Loo, MD Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Kristina Collins, MD, to the development and writing of this article.

References
  1. Macaulay WL. Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant. Arch Dermatol. 1968 Jan. 97(1):23-30. [Medline].

  2. Jaffe ES. The 2008 WHO classification of lymphomas: implications for clinical practice and translational research. Hematology Am Soc Hematol Educ Program. 2009. 523-31. [Medline].

  3. Kempf W, Sander CA. Classification of cutaneous lymphomas - an update. Histopathology. 2010 Jan. 56 (1):57-70. [Medline].

  4. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011 May 12. 117 (19):5019-32. [Medline].

  5. Slater DN. The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants. Br J Dermatol. 2005 Nov. 153(5):874-80. [Medline].

  6. Willemze R, Meijer CJ. Classification of cutaneous T-cell lymphoma: from Alibert to WHO-EORTC. J Cutan Pathol. 2006 Feb. 33 Suppl 1:18-26. [Medline].

  7. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005 May 15. 105(10):3768-85. [Medline].

  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. 2011 Oct 7. [Medline].

  9. Rassidakis GZ, Thomaides A, Atwell C, et al. JunB expression is a common feature of CD30+ lymphomas and lymphomatoid papulosis. Mod Pathol. 2005 Oct. 18(10):1365-70. [Medline].

  10. Clarke LE, Bayerl MG, Bruggeman RD, et al. Death receptor apoptosis signaling mediated by FADD in CD30-positive lymphoproliferative disorders involving the skin. Am J Surg Pathol. 2005 Apr. 29(4):452-9. [Medline].

  11. Pujol RM, Muret MP, Bergua P, Bordes R, Alomar A. Oral involvement in lymphomatoid papulosis. Report of two cases and review of the literature. Dermatology. 2005. 210(1):53-7. [Medline].

  12. de-Misa RF, Garcia M, Dorta S, et al. Solitary oral ulceration as the first appearance of lymphomatoid papulosis: a diagnostic challenge. Clin Exp Dermatol. 2009 May 21. [Medline].

  13. Wang HH, Lach L, Kadin ME. Epidemiology of lymphomatoid papulosis. Cancer. 1992 Dec 15. 70(12):2951-7. [Medline].

  14. Moura FN, Thomas L, Balme B, Dalle S. Dermoscopy of lymphomatoid papulosis. Arch Dermatol. 2009 Aug. 145(8):966-7. [Medline].

  15. Kim YC, Yang WI, Lee MG, et al. Epstein-Barr virus in CD30 anaplastic large cell lymphoma involving the skin and lymphomatoid papulosis in South Korea. Int J Dermatol. 2006 Nov. 45(11):1312-6. [Medline].

  16. Vonderheid EC, Kadin ME, Gocke CD. Lymphomatoid papulosis followed by pityriasis lichenoides: a common pathogenesis?. Am J Dermatopathol. 2011 Dec. 33(8):835-40. [Medline].

  17. Zheng Y, Jia J, Tian Q, Dong X, Wang X, Ying Z, et al. Lymphomatoid papulosis misdiagnosed as pityriasis lichenoides et varioliformis acuta: Two case reports and a literature review. Exp Ther Med. 2014 Dec. 8(6):1927-1933. [Medline]. [Full Text].

  18. Leo AM, Ermolovich T. Lymphomatoid papulosis while on efalizumab. J Am Acad Dermatol. 2009 Sep. 61(3):540-1. [Medline].

  19. Talsania N, O'Toole EA. Severe hypersensitivity reaction to minocycline in association with lymphomatoid papulosis. Clin Exp Dermatol. 2009 Oct. 34(7):e397-8. [Medline].

  20. KIM SK, KIM YC. Lymphomatoid papulosis after allogenic stem cell transplantation. Eur J Dermatol. 2009 Sep-Oct. 19(5):520-1. [Medline].

  21. 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 Oct 13. 118(15):4024-35. [Medline]. [Full Text].

  22. 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 Apr. 140(4):441-7. [Medline].

  23. Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol. 1993 Jun. 28(6):973-80. [Medline].

  24. Liu HL, Hoppe RT, Kohler S, Harvell JD, Reddy S, Kim YH. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol. 2003 Dec. 49(6):1049-58. [Medline].

  25. 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 Feb. 41(2):88-100. [Medline].

  26. Kempf W, Kazakov DV, Schärer L, Rütten A, Mentzel T, Paredes BE, et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol. 2013 Jan. 37(1):1-13. [Medline].

  27. 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 Aug. 37(8):1173-81. [Medline].

  28. Bergstrom JS, Jaworsky C. Topical methotrexate for lymphomatoid papulosis. J Am Acad Dermatol. 2003 Nov. 49(5):937-9. [Medline].

  29. Vonderheid EC, Sajjadian A, Kadin ME. Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. J Am Acad Dermatol. 1996 Mar. 34(3):470-81. [Medline].

  30. Jain N, Gutte R, Jadhav P, Khopkar U. Granulomatous and eccrinotropic lymphomatoid papulosis. Indian J Dermatol Venereol Leprol. 2012 Jan. 78(1):82-4. [Medline].

  31. Yip L, Darling S, Orchard D. Lymphomatoid papulosis in children: experience of five cases and the treatment efficacy of methotrexate. Australas J Dermatol. 2011 Nov. 52(4):279-83. [Medline].

  32. Newland KM, McCormack CJ, Twigger R, Buelens O, Hughes CF, Lade S, et al. The efficacy of methotrexate for lymphomatoid papulosis. J Am Acad Dermatol. 2015 Jun. 72 (6):1088-90. [Medline].

  33. Bruijn MS, Horváth B, van Voorst Vader PC, Willemze R, Vermeer MH. Recommendations for treatment of lymphomatoid papulosis with methotrexate: a report from the Dutch Cutaneous Lymphoma Group. Br J Dermatol. 2015 May 22. [Medline].

  34. Hoetzenecker W, Guenova E, Hoetzenecker K, Yazdi A, Röcken M, Berneburg M. Successful treatment of recalcitrant lymphomatoid papulosis in a child with PUVA-bath photochemotherapy. Eur J Dermatol. 2009 Nov-Dec. 19(6):646-7. [Medline].

  35. Hughes PS. Treatment of lymphomatoid papulosis with imiquimod 5% cream. J Am Acad Dermatol. 2006 Mar. 54(3):546-7. [Medline].

  36. Kontos AP, Kerr HA, Malick F, Fivenson DP, Lim HW, Wong HK. 308-nm excimer laser for the treatment of lymphomatoid papulosis and stage IA mycosis fungoides. Photodermatol Photoimmunol Photomed. 2006 Jun. 22(3):168-71. [Medline].

  37. Rodrigues M, McCormack C, Yap LM, et al. Successful treatment of lymphomatoid papulosis with photodynamic therapy. Australas J Dermatol. 2009 May. 50(2):129-32. [Medline].

  38. Beljaards RC, Willemze R. The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol. 1992 Jun. 126(6):596-602. [Medline].

  39. Kempf W, Levi E, Kamarashev J, et al. Fascin expression in CD30-positive cutaneous lymphoproliferative disorders. J Cutan Pathol. 2002 May. 29(5):295-300. [Medline].

 
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Lymphomatoid papulosis type C on the upper back of a 65-year-old woman with waxing and waning papulonodular eruptions for almost 10 years. The eruption was suppressed completely using methotrexate.
Crusted ulcerated papule of lymphomatoid papulosis on the left hip of a 47-year-old woman with a longer than 20-year history of recurrent papulonodular eruption with spontaneous resolution.
Large indurated plaques of anaplastic large cell lymphoma of 2-months' duration on the left lateral thigh of a 57-year-old man with a 5-year history of lymphomatoid papulosis. The lymphomatoid papulosis skin lesions (not pictured) were rarely larger than 6 mm.
Lymphomatoid papulosis type A showing a cluster of pink papules and 2 crusted papules that resolved spontaneously in the left axilla of a 68-year-old man. The first symptoms developed in the popliteal fossa 8 years before erupting into more widespread papules 10 months before this photograph was taken.
 
 
 
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