Serologic Abnormalities in Macular Lymphocytic Arteritis with Case Presentation

Abstract

Macular lymphocytic arteritis (MLA) is an indolent cutaneous small-medium-vessel vasculitis characterized by widespread asymptomatic livedo racemosa. A number of serologic abnormalities have been reported including an elevated erythrocyte sedimentation rate and antibodies associated with antiphospholipid antibody syndrome. We present a case of MLA with multiple serologic abnormalities, including those that have yet to be reported, such as anti-U1 ribonucleotide protein, anti-RNA polymerase III, anti-smith, and anti-proteinase 3 antibodies. We also provide a brief review of this unfamiliar entity with a focus on the appropriate workup.

Keywords: Antiphospholipid antibodies; Lupus autoantibodies; Lymphocytic thrombophilic arteritis; Macular lymphocytic arteritis; Serologic abnormalities.

Fig. 1

Clinical image. Reticulated dark brown macules and patches resembling livedo racemosa on the bilateral lower legs.

Fig. 2

Histopathology image − punch biopsy from the left lower leg.aH&E staining (×15) demonstrates a medium-vessel vasculitis involving a vessel at the deep dermal-subcuticular junction.bH&E staining (×40) demonstrates lymphocytes with very rare neutrophils in the inflammatory infiltrate with luminal fibrin deposition and narrowing of the vessel lumen.cH&E staining (×100) demonstrates a distinct luminal ring of fibrin within the affected vessel. H&E, hematoxylin and eosin.