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

Abstract

Lymphocytic thrombophilic arteritis is a distinctive but poorly understood lymphocytic vasculitis. There is a need to better define this entity. 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. 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. 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. 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.