Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas

Area of Science:

• Dermatology
• Hematology
• Pathology

Background:

• Lymphomatoid papulosis (LyP) is a CD30-positive lymphoproliferative disorder with a variable clinical course.
• Existing classifications delineate four histological types (A-D) of LyP.
• Angioinvasion and ulceration are uncommon in LyP and can mimic aggressive lymphomas.

Purpose of the Study:

• To analyze the clinicopathologic and molecular characteristics of angioinvasive LyP.
• To define a new variant of LyP with distinct clinical and histological features.
• To differentiate this variant from aggressive lymphomas.

Main Methods:

• Retrospective analysis of 16 patients with angioinvasive LyP.
• Review of clinical presentations, histological findings, and treatment outcomes.
• Immunohistochemical analysis for CD30 and CD8 expression.

Main Results:

• Identified a new LyP variant (proposed LyP type E) with oligolesional, rapidly ulcerating papules forming necrotic eschar-like lesions (1-4 cm).
• Histology showed angiocentric, angiodestructive infiltrates of CD30+, CD8+ atypical lymphocytes.
• Lesions exhibited spontaneous regression, common recurrences, but an excellent prognosis with no extracutaneous spread or deaths.
• Complete remission achieved in 56% of patients.

Conclusions:

• Angioinvasive LyP represents a distinct variant (LyP type E) characterized by aggressive-looking lesions but a favorable prognosis.
• This variant requires differentiation from aggressive angiocentric and angiodestructive T-cell lymphomas.
• LyP type E highlights the spectrum of CD30-positive lymphoproliferative disorders.