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Livedo Reticularis: an Enigma Shirish R REVIEWS IMAJ • VOL 17 • FebruAry 2015 Livedo Reticularis: An Enigma Shirish R. Sangle MBBS MD and David P. D’Cruz MD FRCP Lupus Research Unit, Guy’s and St Thomas’ Hospital NHS Foundation Trust and Kings College Medical School, London, UK livedoid vasculitis is used for cutaneous ulcers with livedo and KEY WORDS: livedo reticularis, thrombosis, pregnancy morbidity, is generally of the racemosa type [4]. accelerated atherosclerosis, livedoid vasculopathy Certain drugs such as amantadine used in the treatment of IMAJ 2015; 17: 104–107 Parkinson’s disease and multiple sclerosis can induce livedo, which may occur in up to 28% of patients receiving aman- tadine. Skin biopsies of livedo in these patients did not show any vasculitis and it was thought to be secondary to depleted ivedo reticularis, as the name suggests, is a livedoid discol- catecholamines [5]. L oration of the skin in a reticular pattern. In 1907 Ehrmann described two types of livedo: livedo racemosa which has an incomplete (broken) reticular lattice work pattern, and livedo PREVALENCE reticularis where the reticular pattern comprises complete circles The first description of an association between livedo reticularis (unbroken) [1]. The concept was that pathological livedo was and cerebrovascular accidents came from Sneddon in 1965 [6]. racemosa and the reticularis pattern was non-pathological. He described six patients, one man and five women, with cerebro- However, in clinical practice there is no clear-cut differentiation vascular accidents who had livedo reticularis. All patients were between these forms of livedo, negative for LE cells and had and often “livedo reticularis” Livedo reticularis is an independent marker no clinical features of systemic is the term used to describe all of arterial and venous thrombosis, lupus erythematosus (SLE) or types of livedo. pregnancy morbidity, and possibly polyarteritis nodosa, although Broadly speaking, livedo is accelerated atherosclerosis none appear to have been tested divided into physiological and for the lupus anticoagulant. pathological livedo. Physiological livedo (cutis marmorata) is Interestingly, most of the patients were hypertensive and one commonly seen on the legs of young healthy women in cold patient underwent a renal biopsy which showed hypertensive weather and varies according to temperature (rewarming). This changes [6]. Livedo is also observed in various autoimmune lattice-like pattern is a result of livedoid discolouration due to diseases such as livedoid vasculopathy, SLE with or without anastomoses between vascular cones in the skin where desatu- antiphospholipid (Hughes) syndrome (APS), thromboangiitis rated blood stagnates [2] [Figure 1]. obliterans, primary thrombocytopenia, poly- The classification of pathological livedo Figure 1. Livedo reticularis arteritis nodosa, and polycythemia vera [7]. was not clear until recently. However, in the Although livedo may present in isolation as revised classification criteria for antiphospho- seen in Sneddon’s syndrome, there is a strong lipid syndrome (APS), pathological livedo is correlation between APS and livedo. In his first defined as “the persistent, non-reversible with description of APS, Hughes included livedo as rewarming, violaceous, red and blue, reticular part of a syndrome associated with arterial and or mottled pattern of the skin of trunk, arms venous thrombosis, spontaneous abortions, or legs, consisting of regular unbroken circles neurological manifestations, thrombocytopenia (livedo reticularis) or irregular broken pattern and livedo [8]. Livedo is the most commonly (racemosa).” Here the concept was based on the observed cutaneous lesion in APS [9]. In their extent and fixed pattern of livedo rather than study of 200 APS patients, Frances et al. [10] the type of livedo. This was further classified found livedo in 25.5%. Livedo can be an initial according to the width of livedo (< or > 10 mm) manifestation in primary APS in up to 40% of – large and fine livedo racemosa and large and patients and more frequently in up to 70% of fine livedo reticularis. It is not clear whether the patients with SLE and APS [11]. Similar find- width of the livedo has any implications for the ings were described in a cohort of 1000 patients observed pathology in the skin [3]. The term with APS. In this cohort, livedo was observed in 104 IMAJ • VOL 17 • FebruAry 2015 REVIEWS 20.4% of patients and was significantly more prevalent in women • livedoid vasculitis (LV) (26% vs. 16%) than in men [12]. Livedo may present as a cutaneous ulcer (livedoid vasculopathy, There is a strong association between livedo and anticardio- LV), also known as livedo reticularis ulcerations, and atrophie lipin antibodies (aCL). Weinstein and co-authors [13] found blanche may be a consequence. This usually affects the legs livedo in almost 50% of patients with SLE. In this cohort of bilaterally and is more common in summer [20-22]. It may 78 patients there was significant correlation between immu- affect all age groups but is more common in young females. noglobulin G (IgG) aCL and severe to moderate livedo. On Initially, it starts with a painful papular rash that develops into the other hand, the prevalence of the lupus anticoagulant was an ulcer. It subsequently heals, leaving a porcelain white scar not significantly different between the livedo and non-livedo that is often star-shaped: atrophie blanche [23]. LV was origi- groups [13]. nally described as a clinical manifestation of vasculitis; however, the present concept is that it is a vaso-occlusive phenomenon with thrombosis of intradermal venules [24,25]. LV has been CLINICAL ASPECTS described as secondary LV when associated with a coagulation • ARTERIAL AND VENOUS THROMBOSIS defect otherwise known as primary or idiopathic. Arterial and venous thrombosis was observed in patients with livedo irrespective of the presence of antiphospholipid anti- bodies (aPL). The relationship between livedo and stroke was PATHOPHYSIOLOGY first described by Sneddon [6]. Frances et al. [14] described The pathophysiology of livedo is not clearly defined. The 46 patients with livedo of whom 27 were aPL negative. They results of skin biopsy depend on the site of the biopsy. Wohlrab found both arterial and venous thrombosis in both groups and team [26] noted that biopsies from the center of the (positive and negative aPL), and skin biopsies showed arterial livedo lesion (white area) had better yield than other areas. occlusions in both groups [14]. Furthermore, Frances et al. Histological findings of livedo were similar in primary APS reported a significant correlation between livedo and seizures, and APS associated with SLE. cardiac valvular defects, systemic hypertension, and Raynaud’s Zelger et al. [27] described 15 patients with Sneddon’s syn- phenomenon. Our own studies confirmed that renal artery drome of whom 12 had skin biopsies. Only small to medium- stenosis in APS was associated with livedo [15]. In a large series sized arteries of the dermis-subcutis boundary were found to be of 308 patients, Toubi and colleagues [16] noted an increased involved. Lesions follow a distinct course. Zelger et al. described prevalence of migraine, stroke and seizure disorder in patients four stages. An initial phase (stage I), characterized by the with livedo and APS. attachment of lymphohistiocytic cells and detachment of endo- thelial cells (endothelitis), is followed by an early phase (stage • Pregnancy morbidity II), which displays partial or complete occlusion of the lumen by Pregnancy morbidity was also higher in our patients with SLE a plug of lymphohistiocytic cells and fibrin. In an intermediate and livedo who were negative for antiphospholipid antibodies phase (stage III), the occluding plug is replaced by proliferat- [17]. These findings further confirmed The underlying pathology ing subendothelial cells accompanied the observations of Frances et al. [14] by dilated capillaries in the adventitia involves prothrombotic as well as that pregnancy loss is more common in of the occluded vessel. The late phase livedo patients with and without aPL. immunologic processes (stage IV) shows fibrosis and shrinkage Livedo may be independently associated with pregnancy loss of the affected vessels [27]. Sepp and colleagues [28] studied in patients who are negative for aPL. skin specimens of 18 patients with Sneddon’s syndrome, and reported that CD3+, UCHL-1+, and HLA-DR+ cells consti- • accelerated atherosclerosis tuted a significant proportion of the inflammatory infiltrate in A preliminary controlled study in our unit found that ankle bra- the early stages, whereas in later stages, endothelial cells and chial pressure index (ABPI) was abnormal in patients with livedo leukocytes were scarce. These findings indicate the possibility as compared to those without livedo [18]. Taking a clue from of an inflammatory and/or immunological process involved in this finding, we further investigated ABPI, pulse wave velocity the pathogenesis of livedo. There were no significantly different (PWV) and pulse contour analysis in four groups consisting of histological findings of livedo on skin biopsies in primary APS 74 patients with APS, SLE and APS, and patients with livedo and APS associated with SLE [10]. only. In this study, 41 patients had livedo and 33 did not. The In contrast to these findings, histopathological changes PWV was significantly abnormal in the livedo group compared were different in patients with livedoid vasculitis (LV) and to the non-livedo group.
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