Lymphocytic Thrombophilic Arteritis (Macular Lymphocytic Arteritis)

Abstract

Lymphocytic thrombophilic arteritis (LTA)/macular lymphocytic arteritis (MLA), is a clinicopathologic entity first described in 2003, as a new peculiar and indolent skin vasculitis. LTA (MLA) has sometimes long duration before diagnosis. It is characterized by asymptomatic erythematous to hyperpigmented macules on the extremities, without palpable purpura or erythematous nodules, whose histology demonstrates a medium-sized vessel arteritis with lymphocytic infiltrate. Arteries lumina are narrowed by the thick intimal fibrin deposit and may be occluded by a luminal thrombosis. Few data are available regarding the pathogenesis of the disease, and initial biologic workup is often unremarkable. There has been continuous debate regarding LTA (MLA) classification as a distinct disease or as an indolent latent form of cutaneous polyarteritis nodosa (cPAN). Indeed, the reparative stage of cPAN can mimic LTA (MLA) histopathologically. In this context, careful clinicopathologic correlation and clinical follow-up is mandatory to classify a patient as having LTA (MLA) or cPAN.