Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity

Abstract

Lymphomatoid papulosis (LyP) is currently categorized as a primary lymphoproliferative disorder that follows a chronic, recurrent clinical course. The diagnosis of LyP is mainly based on clinical presentation and histopathological correlation. Six subtypes of LyP have been described and recognized, each with different histological features and sometimes distinct clinical presentations. LyP type E is a subtype that histologically shows angioinvasion and angiodestruction by CD8 and CD30-positive pleomorphic T cells. Clinically, it usually presents with a few large necrotic nodules or ulcers on the trunk or extremities, unlike other subtypes of LyP. Despite an indolent clinical course, long-term follow-up is necessary due to the risk of developing concurrent or secondary lymphoma. In this report, we demonstrate a case of lymphomatoid papulosis type E presented with widespread small papulonecrotic eruptions, an atypical clinical manifestation, and an unusual immunohistochemical profile. The biopsy revealed CD8, CD30, CD56, and TCR-γ-positive atypical lymphocytic infiltration with angioinvasion and angiodestruction. The patient was successfully treated with low-dose methotrexate.