Clinicopathological features of lymphocytic thrombophilic arteritis and the relationship with livedoid vasculopathy

BACKGROUNDLymphocytic thrombophilic arteritis (LTA) is a distinctive but poorly understood lymphocytic vasculitis. There is a need to better define this entity.

OBJECTIVESTo better characterize the clinicopathological features of LTA.

METHODSWe conducted a case-series analysis of all patients with a diagnosis of LTA known to St Vincent's Hospital Melbourne Dermatology Unit, in Victoria, Australia, a referral centre for cutaneous vasculitis and LTA. 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. Data were collated on demographics, comorbidities, clinical features, histopathology, treatments and clinical response.

RESULTSThirty-six patients were identified including 26 female and 10 male patients with a mean (SD) age of onset of 32.7 (13.0) years. A high proportion of patients of Asian heritage was noted (n = 16, 44%). Most patients (n = 34, 94%) presented with widespread livedo racemosa. Less common manifestations included petechiae (n = 6, 17%), macular pigmentation (n = 8, 22%), neuropathy (n = 12, 33%) and ulceration (n = 12, 33%). A significant association was noted between ulceration and superficial dermal vessel involvement on biopsy, similar to livedoid vasculopathy. All patients had a neutrophil-poor lymphocytic perivascular infiltrate with fibrinoid change in the deeper dermis/upper subcutis. A variety of vessels were affected; 44% (n = 16) involved vessels in the superficial dermis as well as the deep dermis/upper subcutis. A discrete concentric fibrin ring (n = 15, 42%) was not essential for diagnosis. Treatments targeting vascular fibrin deposition appeared effective for all manifestations other than livedo which remained persistent.

CONCLUSIONSLTA has distinctive clinicopathological features and may lie on a spectrum with livedoid vasculopathy. Although livedo racemosa persists, other manifestations respond to antithrombotic treatments. An author video to accompany this article is available online.

Abstract from PubMed