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Macular lymphocytic arteritis
Other Resources UpToDate PubMed

Macular lymphocytic arteritis

Contributors: Vivian Wong MD, PhD, Saagar Jadeja, Susan Burgin MD
Other Resources UpToDate PubMed


Macular lymphocytic arteritis (MLA), also known as lymphocytic thrombophilic arteritis, is a rare medium-vessel lymphocytic vasculitis that affects the skin. It characteristically presents as multiple, asymptomatic, hyperpigmented macules on the lower extremities or as widespread, persistent livedo racemosa. MLA tends to follow an indolent course with no evidence of progression to systemic involvement.

In a review of 32 cases of MLA, the mean age of onset was 40 years, with onset ranging from 6-73 years. Women and children represented 84% and 6% of all cases that were reviewed, respectively. Individuals of African descent represented the largest ethnic group affected by MLA.

The etiology of MLA is unknown. Overlap of some histological features between MLA and certain stages of cutaneous polyarteritis nodosa (C-PAN) have raised the question of whether MLA actually exists as part of a spectrum with C-PAN.


L95.8 – Other vasculitis limited to the skin

46286007 – Lymphocytic vasculitis of skin

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Differential Diagnosis & Pitfalls

  • Capillaritis 
  • Postinflammatory hyperpigmentation 
  • Lichen planus pigmentosus – Most commonly affects sun-exposed areas including the face and neck.
  • Ashy dermatosis – Typically presents as gray-blue macules and patches affecting the trunk and proximal extremities.
  • Drug-induced pigmentation 
Differential diagnosis for livedo racemosa-like presentations:
  • Sneddon syndrome
  • Cutaneous or systemic Polyarteritis nodosa – Typical presentation includes tender subcutaneous nodules with possible ulceration.
  • Granulomatosis with polyangiitis
  • Connective tissue diseases (Systemic lupus erythematosus, Rheumatoid arthritis, Dermatomyositis)
  • Hematologic or hypercoagulable conditions (Antiphospholipid antibody syndrome, Cryoglobulinemia, Essential thrombocythemia, Polycythemia vera, Protein C deficiency or Protein S deficiency, Disseminated intravascular coagulation)

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Last Reviewed:09/02/2020
Last Updated:09/02/2020
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Macular lymphocytic arteritis
A medical illustration showing key findings of Macular lymphocytic arteritis
Copyright © 2024 VisualDx®. All rights reserved.