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Lymphocytic Thrombophilic Arteritis

A Review

Vakili, Sharif, MS*; Zampella, John G., MD; Kwatra, Shawn G., MD*; Blanck, Jaime, MPA; Loss, Manisha, MD*

JCR: Journal of Clinical Rheumatology: April 2019 - Volume 25 - Issue 3 - p 147–152
doi: 10.1097/RHU.0000000000000846
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Macular lymphocytic arteritis or lymphocytic thrombophilic arteritis (LTA) is a recently described cutaneous arteritis that is characterized by asymptomatic macules and patches of the extremities associated with lymphocytic arteritis at the deep dermal/subcuticular junction for which little information exists on demographics, evaluation, and management. There has been recent debate in the literature whether this disease is a new distinct entity, misdiagnosed cutaneous polyarteritis nodosa (cPAN), or a disease on a spectrum with cPAN. We systematically review the literature on demographic information, medical history, histopathology findings, and treatment management to analyze trends and clarify controversies in characterizations of LTA. Forty detailed cases of LTA have been published. We submit that, although literature is limited, a review of the data still suggests that LTA is distinct from cPAN and systemic PAN. In addition, to better reflect the pathophysiologic natural history of this condition and correct for the historical artifact of how the disease was identified, we encourage the disease to be referred to as LTA and discourage ongoing use of macular lymphocytic arteritis.

From the *Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD,

Department of Dermatology, New York University, New York, NY; and

Johns Hopkins University, Welch Library, Baltimore, MD.

The authors declare no conflict of interest.

All authors had substantial contributions in the concept design, interpretation of the data, drafting of the work, and have approved the final version of this article. All agree to be accountable for all aspects of the work.

Sharif Vakili and John G. Zampella contributed equally to this work.

Correspondence: Sharif Vakili, MS, 733 N Broadway, Baltimore, MD 21205. E-mail: sharif.vakili@jhmi.edu.

Supplemental digital content is available for this article. Direct URL citation appears in the printed text and is provided in the HTML and PDF versions of this article on the journal’s Web site (www.jclinrheum.com).

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