Livedoid vasculitis: a manifestation of the antiphospholipid syndrome?

Abstract

Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition.

Livedoid Vasculopathy

“…40 Cutaneous ulceration and necrosis have been described in association with circulating LA, 41 and LA and ACAs have been reported in patients with livedoid vasculopathy. 4,42,43 The prevalence of LA in our study group is higher than that in the healthy population (8%). 44 Similarly, 28.6% of our patients were found to have ACA, compared with 5% to 12% reported in healthy persons, although 1 study reported a 51.6% prevalence of IgG ACA in healthy elderly subjects.…”

Warfarin Therapy for Livedoid Vasculopathy Associated With Cryofibrinogenemia and Hyperhomocysteinemia

“…Although atrophie blanche is a consequence of the lesions of livedoid vasculopathy, it is not pathognomonic of this disorder and can occur in association with other conditions such as stasis dermatitis and collagen vascular diseases. 2,4,6,7 Although it is quite distinctive from a vasculitis or immune-mediated process, livedoid vasculopathy has been confused with small vessel vasculitis in the past and can be misdiagnosed, as demonstrated by our patient's diagnostic history. The term segmental hyalinizing vasculitis was commonly used to describe livedoid vasculopathy in the 1970s.…”

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

“…The aetiology of the disorder is unknown. Skin biopsy argues against a real vasculitis and shows fibrin deposition within affected walls of vessels and thrombus formation within the lumen [1]. Livedoid vasculopathy seems to be thrombo-occlusive vasculopathy.…”

“…It can be associated with systemic disorders like SLE or present in an "ideopathic" form [60]. A number of studies report about different coagulation abnormalities: elevated fibrinopeptid A [62], defective release of vascular plasminogen activator [73], decreased thrombomodulin expression [100] and protein C deficiency [4].Acland et al have suggested that livedoid vasculopathy may be a manifestation of antiphospholipid syndrome [1].…”