Clinicopathological features of lymphocytic thrombophilic arteritis and the relationship with livedoid vasculopathy - a case series of 36 patients

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ID: 319581
2026
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Abstract
BACKGROUND: Lymphocytic thrombophilic arteritis is a distinctive but poorly understood lymphocytic vasculitis. There is a need to better define this entity. OBJECTIVE: We conducted a case-series analysis of all patients with a diagnosis of lymphocytic thrombophilic arteritis known to St Vincent's Hospital Melbourne Dermatology Unit, in Victoria, Australia, a referral centre for cutaneous vasculitis and lymphocytic thrombophilic arteritis, to better characterise the clinicopathological features of this entity. METHODS: All patients who met our clinicopathological criteria for this condition were included. Diagnostic clinical features included the presence of widespread blanchable livedo racemosa and/or macular pigmentation. Diagnostic histological features included the presence of a lymphocytic arteritis with fibrin deposition affecting vessels in the deeper dermis and/or upper subcutis.Cases of lymphocytic thrombophilic arteritis were assessed for demographics, comorbidities, clinical features, histopathology, treatments, and clinical response. RESULTS: Thirty-six cases were identified including 26 female and 10 male patients with an average age of onset of 32.7 years. A high proportion of cases with Asian descent was noted (44.4%). Most cases (94.4%) presented with widespread livedo racemosa. Less common manifestations included petechiae (16.7%), macular pigmentation (22.2%), neuropathy (33.3%), and ulceration (33.3%). A significant association was noted between ulceration and superficial dermal vessel involvement on biopsy, similar to livedoid vasculopathy.All cases demonstrated a neutrophil-poor lymphocytic perivascular infiltrate with fibrinoid change in the deeper dermis/upper subcutis. A variety of vessels were affected; 44.4% involved vessels in the superficial dermis as well as the deep dermis/upper subcutis. A discrete concentric fibrin ring (41.7%) was not essential for diagnosis.Treatments targeting vascular fibrin deposition appeared effective for all manifestations other than livedo for which no cases responded. CONCLUSION: Lymphocytic thrombophilic arteritis has distinctive clinicopathological features and may lie on a spectrum with livedoid vasculopathy. Although livedo racemosa persists, other manifestations respond to antithrombotic treatments.
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Authors Christian Gan, Edmund Wee, Daryl Johnson, Robert Kelly
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Journal the british journal of dermatology
Year 2026
DOI
10.1093/bjd/ljag273
URL
https://doi.org/10.1093/bjd/ljag273
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