Lymphocytic thrombophilic arteritis complicated by systemic involvement

Abstract

Lymphocytic thrombophilic arteritis (LTA) is a recently described entity defined by primary lymphocytic vasculitis; it typically has a chronic indolent course. We describe a patient who presented with clinical and histological findings consistent with LTA and later developed bilateral focal testicular infarcts as well as an acute median nerve neuropathy.

Serologic Abnormalities in Macular Lymphocytic Arteritis with Case Presentation

“…CPAN, in contrast, was associated with localized starburst livedo, purpura, episodes of pain, nodules and inflammatory ulcerations, and the presence (≥5%) of neutrophils on histology [6]. While there has been a single report of a patient diagnosed as MLA with evidence of systemic vasculitis (testicular infarcts and mononeuritis) [9], this disease overall follows a persistent indolent course. There have also been no reports of MLA/LTA with progression to cPAN, which also supports consideration of MLA/LTA and cPAN as distinct entities.…”

“…This includes patients with other risk factors such as smoking and pregnancy. Possible exceptions to this include the single report of systemic involvement with testicular infarction [9] and 1 patient who developed transient vision loss in 1 eye lasting 2 min [13]. Criteria for the diagnosis of MLA/LTA has been posed as: (1) presence of macules, papules, and/or patches that follow a benign course; (2) histopathology demonstrating small-medium-vessel vasculitis with a predominantly lymphocytic infiltrate; and (3) absence of signs or symptoms to suggest systemic vasculitis [4].…”

“…In most reports, these lesions are non-indurated, and nodules or ulcerations are absent on physical exam. Atypical clinical presentations including annular morphology, unilateral or diffuse distribution of lesions, the presence of nodules, ulcerations, or systemic involvement such as testicular infarction and neuropathy have been reported [6][7][8][9][10]. MLA disproportionately affects women, primarily of black ethnicity, with the second most common groups being Asian and Caucasian patients [4].…”

“…HBV 1 ANA, antinuclear antibody; RF, rheumatoid factor; ANCA, antineutrophil cytoplasmic antibody; RNP, ribonucleotide protein; CCP, cyclic citrullinated peptide; HIV, human immunodeficiency virus; HBV, hepatitis B virus. of systemic involvement with testicular infarction [9] and 1 patient who developed transient vision loss in 1 eye lasting 2 min [13]. Criteria for the diagnosis of MLA/LTA has been posed as:…”

Three cases of Sneddon syndrome: A comparison with lymphocytic thrombophilic arteritis

“…Lymphocytic thrombophilic arteritis was initially described in 2004 as a lymphocytic arteritis that most commonly presents with widespread livedo racemosa 2 . Other cutaneous manifestations include macular pigmentation, ulceration and peripheral neuropathy including mononeuritis multiplex 4‐6 . In most cases, however, persistent livedo racemosa was the only cutaneous finding 3 .…”