eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Lymphomatoid Papulosis: Treatment & Medication

Author: John A Zic, MD, Director of Cutaneous Lymphoma Clinic, Assistant Professor, Division of Dermatology, Vanderbilt University Medical Center
Contributor Information and Disclosures

Updated: Mar 20, 2008

Treatment

Medical Care

  • In the past, localized mildly pruritic skin lesions were treated with mid- to high-potency topical steroids to hasten resolution. Many authorities currently are more inclined to treat lesions with systemic or more aggressive topical therapies, including phototherapy, to suppress the disease and the possibility of progression to MF, ALCL, or HD.
  • Low-dose weekly methotrexate (MTX)8,9 is a safe and effective treatment for suppressing LyP; however, the disease recurs within 1-2 weeks after discontinuing the medication.
  • Oral psoralen plus UVA (PUVA) phototherapy also effectively treats and suppresses the disease.
  • A few reports also have found that topical carmustine, topical nitrogen mustard, topical MTX, topical imiquimod cream,10 intralesional interferon, low-dose cyclophosphamide, chlorambucil, medium-dose UVA-1 therapy, excimer laser therapy,11 and dapsone help disease suppression.

Consultations

  • Dermatologist: Consultation is recommended for evaluating clinical findings and obtaining skin biopsy specimens of appropriate lesions. Ideally, consult a dermatologist with experience in the management of cutaneous lymphomas.
  • Dermatopathologist: Consultation is recommended for histologic evaluation of skin biopsy specimens, with occasional consultation by a hematopathologist for patients with borderline biopsy results.

Activity

LyP requires no activity restrictions.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Antimetabolites

Inhibit cell growth and proliferation by blocking key steps of the cell cycle.


Methotrexate (Folex, Rheumatrex)

Antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells; may suppress immune system. First-line oral agent for treatment of LyP. Disease usually is sensitive to low, weekly oral doses. Adjust dose gradually to attain satisfactory response.

Adult

5-10 mg PO qwk; may require up to 25 mg/wk to respond

Pediatric

Not established; 2.5-5 mg PO qwk suggested

Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; folic acid or derivatives contained in some vitamins may decrease response to MTX; coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides (including TMP-SMZ) may increase effects and toxicity of MTX; may increase plasma levels of thiopurines

Documented hypersensitivity; alcoholism; hepatic insufficiency; immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); breastfeeding

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Monitor CBC counts monthly and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk exists of elevated MTX levels, eg, dehydration); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood cell counts occurs; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested); may cause mucositis or cutaneous ulceration

Phototherapy

Induce apoptosis in activated T cells. PUVA phototherapy effectively treats and suppresses disease.


Methoxsalen (8-MOP, Oxsoralen)

Inhibits mitosis by binding covalently to pyrimidine bases in DNA when photoactivated by UVA. In Europe, this modality is more popular than MTX for treating LyP. Available in 10-mg cap.

Adult

0.4 mg/kg/dose PO 1.5 h prior to UVA exposure; alternatively, 0.57 mg/kg 1.5-2 h before exposure to UVA, at least 48 h apart

Pediatric

Administer as in adults

Toxicity increases with phenothiazines, griseofulvin, nalidixic acid, tetracyclines, thiazides, and sulfanilamide

Documented hypersensitivity; squamous cell cancer; cataract; light-sensitive diseases such as lupus or porphyria; ingestion of photosensitizing drugs; hepatitic disease; arsenic therapy; history of melanoma; patients with aphakia

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Severe burns may occur from sunlight or UVA if dose or treatment frequency exceeded; use only if response to other forms of therapy is inadequate; long-term use may increase risk of skin cancer; ocular changes may occur

More on Lymphomatoid Papulosis

Overview: Lymphomatoid Papulosis
Differential Diagnoses & Workup: Lymphomatoid Papulosis
Treatment & Medication: Lymphomatoid Papulosis
Follow-up: Lymphomatoid Papulosis
Multimedia: Lymphomatoid Papulosis
References
[ CLOSE WINDOW ]

References

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

  2. Slater D. 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;153:874-880. [Medline].

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

  4. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005;105(10):3768-85. [Medline].

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

  6. 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].

  7. 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. Dec 2003;49(6):1049-58. [Medline].

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

  9. 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. Mar 1996;34(3):470-81. [Medline].

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

  11. 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. Jun 2006;22(3):168-71. [Medline].

  12. Kempf W, Levi E, Kamarashev J, Kutzner H, Pfeifer W, Petrogiannis-Haliotis T, et al. Fascin expression in CD30-positive cutaneous lymphoproliferative disorders. J Cutan Pathol. May 2002;29(5):295-300. [Medline].

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

  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. Feb 1 1995;122(3):210-7. [Medline].

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

  16. Demierre MF, Goldberg LJ, Kadin ME, Koh HK. Is it lymphoma or lymphomatoid papulosis?. J Am Acad Dermatol. May 1997;36(5 Pt 1):765-72.

  17. El Shabrawi-Caelen L, Kerl H, Cerroni L. Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C. Arch Dermatol. Apr 2004;140(4):441-7. [Medline].

  18. el-Azhary RA, Gibson LE, Kurtin PJ, Pittelkow MR, Muller SA. Lymphomatoid papulosis: a clinical and histopathologic review of 53 cases with leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene rearrangement studies. J Am Acad Dermatol. Feb 1994;30(2 Pt 1):210-8. [Medline].

  19. Flann S, Orchard GE, Wain EM, Russell-Jones R. Three cases of lymphomatoid papulosis with a CD56+ immunophenotype. J Am Acad Dermatol. Nov 2006;55(5):903-6. [Medline].

  20. Franchina M, Kadin ME, Abraham LJ. Polymorphism of the CD30 promoter microsatellite repressive element is associated with development of primary cutaneous lymphoproliferative disorders. Cancer Epidemiol Biomarkers Prev. May 2005;14(5):1322-5. [Medline].

  21. Gellrich S, Wernicke M, Wilks A, Lukowsky A, Muche JM, Jasch KC, et al. The cell infiltrate in lymphomatoid papulosis comprises a mixture of polyclonal large atypical cells (CD30-positive) and smaller monoclonal T cells (CD30-negative). J Invest Dermatol. Mar 2004;122(3):859-61. [Medline].

  22. Greisser J, Doebbeling U, Roos M, Mueller B, Schmid M, Burg G, et al. Apoptosis in CD30-positive lymphoproliferative disorders of the skin. Exp Dermatol. May 2005;14(5):380-5. [Medline].

  23. Gruber R, Sepp NT, Fritsch PO, Schmuth M. Prognosis of lymphomatoid papulosis. Oncologist. Sep 2006;11(8):955-7; author reply 957. [Medline].

  24. Kadin ME. Pathobiology of CD30+ cutaneous T-cell lymphomas. J Cutan Pathol. Feb 2006;33 Suppl 1:10-7. [Medline].

  25. Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol. Feb 2006;33 Suppl 1:58-70. [Medline].

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

  27. 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. Dec 2003;49(6):1049-58. [Medline].

  28. Magro CM, Crowson AN, Morrison C, Merati K, Porcu P, Wright ED. CD8+ lymphomatoid papulosis and its differential diagnosis. Am J Clin Pathol. Apr 2006;125(4):490-501. [Medline].

  29. Schultz JC, Granados S, Vonderheid EC, Hwang ST. T-cell clonality of peripheral blood lymphocytes in patients with lymphomatoid papulosis. J Am Acad Dermatol. Jul 2005;53(1):152-5. [Medline].

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

  31. 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. Jun 1993;28(6):973-80. [Medline].

  32. Wood GS, Crooks CF, Uluer AZ. Lymphomatoid papulosis and associated cutaneous lymphoproliferative disorders exhibit a common clonal origin. J Invest Dermatol. Jul 1995;105(1):51-5. [Medline].

  33. Yagi H, Seo N, Ohshima A, Itoh T, Itoh N, Horibe T, et al. Chemokine receptor expression in cutaneous T cell and NK/T-cell lymphomas: immunohistochemical staining and in vitro chemotactic assay. Am J Surg Path. Sept 2006;30(9):1111-19. [Medline].

  34. Yamaguchi T, Ohshima K, Karube K, Kawano R, Nakayama J, Suzumiya J, et al. Expression of chemokines and chemokine receptors in cutaneous CD30+ lymphoproliferative disorders. Br J Dermatol. May 2006;154(5):904-909. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Macaulay disease, Macaulay's disease, LyP, T-cell lymphoma, T cell lymphoma, cutaneous lymphoma, mycosis fungoides, MF, Hodgkin disease, Hodgkin lymphoma, Hodgkin's disease, Hodgkin's lymphoma, HD, lymphoproliferative disease, lymphoproliferative disorder, LPD, anaplastic large cell lymphoma, ALCL, primary cutaneous anaplastic large cell lymphoma, pcALCL

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

John A Zic, MD, Director of Cutaneous Lymphoma Clinic, Assistant Professor, Division of Dermatology, Vanderbilt University Medical Center
John A Zic, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; 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.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

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 and Association of Professors of Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

RELATED EMEDICINE ARTICLES
RELATED MEDSCAPE ARTICLES
Articles
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.