eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Lymphomatoid Papulosis: Treatment & Medication

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

Updated: Dec 16, 2009

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 Hodgkin disease (HD), anaplastic large cell lymphoma (ALCL), or mycosis fungoides (MF).
  • Low-dose weekly methotrexate (MTX)19,20 is a safe and effective treatment for suppressing lymphomatoid papulosis (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.
  • One report describes successful treatment of recalcitrant lymphoma papulosis in using PUVA-bath photochemotherapy in a pediatric patient.21
  • A few reports also have found that topical carmustine, topical nitrogen mustard, topical MTX, topical imiquimod cream,22 intralesional interferon, low-dose cyclophosphamide, chlorambucil, medium-dose UVA-1 therapy, excimer laser therapy,23 photodynamic therapy,24 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

Lymphomatoid papulosis mandates 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
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References

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Further Reading

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Keywords

lymphomatoid papulosis, 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

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Contributor Information and Disclosures

Author

John A Zic, MD, Director of VU Cutaneous Lymphoma Clinic, Assistant Professor of Medicine 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 Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis  investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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