Lymphocytic Thrombophilic Arteritis

Abstract

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.

Asymptomatic livedoid hyperpigmentation in a middle‐aged woman

“…The term LTA was introduced as alternative nomenclature because these pathologic findings were seen in the absence of macules at presentation . A recent review has suggested that it does not belong on the spectrum of cPAN and systemic PAN due to its almost certain indolent course and lack of systemic symptoms, which is exhibited in our patient’s presentation. A recent review suggests the following criteria be considered in making the diagnosis of LTA: (i) presence of macules, papules, and/or patches that follow a benign course, (ii) histology demonstrating small‐medium vessel vasculitis with a predominantly lymphocytic infiltrate, and (iii) absence of signs or symptoms to suggest systemic vasculitis …”

“…Given the patient’s lack of symptoms and associated pathologic findings, we made the diagnosis of lymphocytic thrombophilic arteritis (LTA). LTA typically presents in middle‐aged women as brown or erythematous hyperpigmented macules or livedoid patches on the lower extremities . Median duration of the clinical course is one year, but it has been reported to last up to 15 years .…”

“…Laboratory workup does not play a significant role in diagnosis. Inflammatory markers and autoimmune markers may be positive, but they do not correlate with presence of symptoms or disease duration . There has been one reported case in a patient with hepatitis B and HIV.…”

“…Typical treatment for cPAN is conservative and usually responds with NSAIDs, colchicine, or systemic corticosteroids for refractory and severe cases. LTA does not require treatment, and its response to various therapies such as dapsone and other immunomodulators has been unremarkable …”

Macular arteritis in an HIV-infected patient improving with adherence to antiretroviral therapy

“…There has been debate in the literature about whether macular arteritis represents an indolent nonnodular variant of cutaneous polyarteritis nodosa or a distinct entity. 4 , 15 , 16 , 17 , 18 , 19 , 20 , 21 …”