Wednesday, October 13, 2010
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.
- Superpotent topical steroids twice daily to new lesions,
followed by pulse therapy on weekends, may be effective and has
- Bexarotene gel 1% may be applied to early lesions as tolerated
once or twice daily. This may prevent progression and speed
- 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.
- PUVA may be effective in controlling LyP.
- Interferon alpha, 3 million units 3 times weekly, may be used
in refractory cases.
- 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.
- 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.
- Oral bexarotene may be associated with hypothyroidism as well
as all the standard retinoid toxicities discussed in the chapter on