Background: Lymphocytic thrombophilic arteritis (LTA), or macular lymphocytic arteritis, is defined by a primary lymphocytic vasculitis. However, the nosology of LTA has been controversial, with speculation that it may represent an indolent non-nodule-forming variant of cutaneous polyarteritis nodosa (cPAN).
Objective: This study compares the clinicopathologic features of patients with LTA or cPAN to assess if these conditions should be considered distinct entities.
Methods: This is a cross-sectional study of all LTA and cPAN cases at a single tertiary center using prospectively collected clinical data and blinded histologic assessment.
Results: The study included 17 patients with LTA and 13 patients with cPAN. Clinically, cases of LTA were distinguished by a more widespread pattern of livedo racemosa, which was noninfiltrated and asymptomatic. In contrast, cPAN was associated with localized starburst livedo, purpura, and episodic features including nodules, pain, and large inflammatory ulcers. When patients were separated according to the presence (>5%) or paucity (≤5%) of neutrophils on blinded histology review, they had distinct clinical features and differences in disease course.
Limitations: This was a single-center study.
Conclusion: Our data support the classification of LTA and cPAN as separate entities rather than a spectrum of the same disorder and highlight the importance of clinicopathologic correlation in distinguishing these conditions.
Keywords: cutaneous polyarteritis nodosa; livedo racemosa; lymphocytic thrombophilic arteritis; lymphocytic vasculitis.
Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.