An Unusual Type of Lymphomatoid Papulosis Presenting in Pregnancy: Risk-Benefit Analysis of Antepartum Treatment [18H

Abstract

INTRODUCTION: Lymphomatoid papulosis (LyP) has an incidence of 2 per 1,000,000 and is associated with lymphoproliferative disorders. Type E, characterized by angioinvasion, is the rarest subtype and is associated with CD30 and CD8 expression. LyP is associated with immune compromising disorders, making pregnancy a potential setting for presentation. We present one of the first cases of LyP type E in pregnancy. METHODS: Case: 30-year-old G5P3, with a pregnancy complicated by a history of hemolysis, elevated liver enzymes and low platelet count syndrome in a previous pregnancy, presented in early pregnancy with a two month history of diffuse painful skin lesions. As the pregnancy progressed, the lesions spread and would blister and ulcerate before resolving. Biopsy returned with CD30+ angioinvasive LyP type E. Prednisone and topical steroids provided minimal symptomatic improvement. Peginterferon alfa-2a was offered as an alternative therapy; however, was deferred until after delivery. CONCLUSION: Appropriate diagnosis of LyP and subtype as well as multi-disciplinary follow-up is essential given possible associated lymphoproliferative disorders. Treatment may involve more risk than benefit and has not been proven to prevent associated malignancy necessitating individualized risk-benefit analysis. Conservative treatment is preferred, even in significantly symptomatic pregnant patients. In some severely symptomatic patients, peginterferon alfa-2a may be considered with a better safety profile compared to other agents in pregnancy. The angiodestructive nature of type E may be associated with pregnancy complications including those on the preeclampsia spectrum. These pregnancies may require closer monitoring and the consideration of aspirin prophylaxis. Early diagnostic biopsy will allow for timely intervention.