LYMPHOMATOID PAPULOSIS

By Gary M. White, MD

Lymphomatoid papulosis Solitary or multiple small red papules are typical.


Lymphomatoid papulosis (LyP) is a primary cutaneous CD30+ lymphoproliferative disorder. It represents a clonal proliferation of T-cells and there is a risk of developing systemic lymphoma (e.g., CTCL, Hodgkin's disease, immunoblastic lymphoma, anaplastic large cell lymphoma). In fact, some experts consider LyP a low-grade malignant cutaneous T-cell lymphoma. Some cases are borderline between lymphomatoid papulosis and CD30 (Ki-1)-positive large cell lymphoma. One study found that the mean time from onset to the development of lymphoma was 12 years and that the cumulative risk for developing lymphoma was 80% in patients who have lymphomatoid papulosis for 15 years [Ann Intern Med. 1995 Feb 1;122(3):210-7].

Clinical

The initial lesion is an erythematous to red/brown papule which in days to weeks may become hemorrhagic or necrotic. Multiple, self-healing lesions occurring in crops in an adult is characteristic. The disease may last from weeks to decades. Histology is suggestive of lymphoma. Individual lesions resolve spontaneously in 3-12 weeks which is a defining feature of LyP. Half of patients heal with scarring. The peak incidence is in the 5th decade. Men are more likely to be affected than women.

Pediatric Lymphomatoid Papulosis

LyP may rarely affect children. In an analysis of 251 pediatric cases of LyP [Am J Clin Dermatol. 2016;17:319], the mean age of onset was 9 years and approximately 5% developed lymphoma. Thus, lifelong follow-up and proper patient counseling are warranted.

Workup

Workup should include routine histology, immunohistochemistry, and T-cell receptor gene rearrangement analysis. Consultation and/or referral to an oncologist may be in order. Various histologic subtypes (e.g., A-D) have been described. Of note, patients with histologic subtype D are less likely to develop secondary lymphomas. LyP type B resembles mycosis fungoides with variable CD30 expression and an epidermotropic infiltrate of small to medium-sized lymphocytes with atypical chromatin-dense nuclei.

Secondary malignancies are common. In one study [Weiser et al, JAAD 2016;74;59], 52% had one or more secondary malignancies before, concomitant to, or after LyP diagnosis. 26% of patients developed hematologic malignancies (CTCL the most common). Interestingly, patients with LP and CTCL have a better prognosis than those with CTCL alone.

Treatment

All patients require long-term followup to monitor for development of lymphoma and most patients with mild disease merely require "watchful waiting." Unfortunately, currently there is no known way to decrease the risk of developing lymphoma in patients with LyP. Patients should undergo a biopsy periodically, and especially if the character or frequency of appearance of lesions accelerates. (For example, a patient who typically gets 4-5 small red papules a month each lasting 5-6 weeks suddenly develops a nodule. That nodule should be biopsied.)

Specific treatment is not needed. However, symptomatic relief or an attempt to induce regression are appropriate reasons to initiate therapy. A potent topical steroid may help speed resolution of lesions and is excellent first line therapy. Some patients with recurrent herpes simplex have noted this to flare the LyP. Thus, an oral antiviral agent, e.g. acyclovir is a good option here. Topical bexarotene and imiquimod have been used.

Oral methotrexate (5–20 mg/week) is an effective therapy to suppress the development of new skin lesions of LyP [BJD 2015;173;1319–1322]. It may be considered in patients with significant pruritus, scarring or widespread disease. Patients may be started at 7.5-10 mg/week along with folic acid and the dose titrated up as needed. Another good option is UVB. Neither radiation nor chemotherapy are recommended.

Brentuximab is a modified humanized antibody to CD30 has been reported helpful in isolated cases [JAAD 2016;74;59]. A study of 12 patients with LyP utilizing intravenous brentuximab vedotin 1.8 mg/kg infused over 30 minutes every 21 days has been reported [JAMA Derm online Nov 2017]. All 12 patients responded to brentuximab vedotin, and 7 exhibited a complete response. Time to response was 3 weeks in all patients. Grade 1 to 2 neuropathy occurred in 10 patients but resolved in 5. Adverse events of grade 3 or higher were neutropenia (n = 2) and dizziness/vertigo (n = 1).

Pediatric

LyP may rarely affect children [PD 2017;34;481]. The clinicopathological presentation does not differ significantly from adults. It should be suspected in the case of a child with recurrent self-healingpapulonodular eruptions with a CD30+lymphoid infiltrate on pathology, One may consider pulse superpotent topical steroids, e.g., clobetasol or halobetasol BID for 1-3 weeks at a time [PD 1996;13;501] or UVB. Lifelong follow up is indicated because of the association with lymphoid malignancies.

References

Localized Lymphomatoid Papulosis. JAAD February 2010 Volume 62, Issue 2, Pages 353–356.

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