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Therapeutic Strategies

Lymphomatoid Papulosis

Timothy Berger Bruce Wintroub

Wednesday, October 13, 2010

Overview

Lymphomatoid papulosis (LyP) is an inflammatory disorder affecting primarily the upper dermal blood vessels. It is characterized by the presence of CD30-positive lymphocytes (sometimes large and atypical) in the dermal infiltrate. Individual lesions resolve spontaneously (albeit over months). Patients with LyP are at increased risk to develop cutaneous T-cell lymphoma and systemic lymphoma of many types. The treatment of LyP does not prevent progression or the development of systemic or cutaneous lymphoma. Treatment should be appropriate for the extent and severity of the disease, as no treatment is curative, only suppressive. Once treatment is stopped, the LyP usually returns to its pretreatment condition. If the patient has a coexistent lymphoma, often chemotherapy will cure the associated lymphoma, but the LyP will recur.

First Steps

  1. Superpotent topical steroids twice daily to new lesions, followed by pulse therapy on weekends, may be effective and has minimal toxicity.
  2. Bexarotene gel 1% may be applied to early lesions as tolerated once or twice daily. This may prevent progression and speed healing.


Alternative Steps

  1. Methotrexate 5-10 mg per week is usually very effective in suppressing lesions of LyP. Therapy usually must be continued to be effective, but less frequent dosing may be possible.
  2. PUVA may be effective in controlling LyP.


Subsequent Steps

  1. Interferon alpha, 3 million units 3 times weekly, may be used in refractory cases.
  2. Oral bexarotene 300 mg/m2 or lower may be used in severe disease when methotrexate is contraindicated or ineffective, and photochemotherapy is not practical or effective. Bexarotene may be combined with PUVA or interferon treatment.


Pitfalls

  1. LyP patients are at increased risk for the development of cutaneous and systemic lymphomas. Routine and regular evaluations are required. Any lesion of LyP that continues to enlarge and does not autoinvolute must be biopsied to rule out the development of anaplastic CD30+ large cell lymphoma.
  2. Oral bexarotene may be associated with hypothyroidism as well as all the standard retinoid toxicities discussed in the chapter on acne.

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