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A study of coagulation and anti-endothelial antibodies in idiopathic livedo reticularis.

Livedo reticularis is associated with collagen vascular diseases and other vaso-occlusive disorders in a substantial number of cases. In the remaining cases the cause of livedo reticularis is still unknown. (i.e., idiopathic). We sought to determine a possible causal relationship between idiopathic livedo reticularis and autoimmune factors associated with the coagulation system, including antiendothelial cell antibodies. Nine patients with idiopathic livedo reticularis were studied. All patients were found to have normal platelet count, fibrinogen levels, and prothrombin and activated partial thromboplastin times, as well as negative results for Venereal Disease Research Laboratory and D-timer tests. Anticoagulant activity was detected in 2 patients: one had positive results of thromboplastin titration index and Russell's viper venom test, as well as increased levels of anticardiolipin antibodies and anti-endothelial cell antibodies; the other has positive thromboplastin titration index, mildly increased levels of anti-endothelial cell antibodies, and markedly increased levels of antinuclear antibodies. A third patient had mildly increased levels of anti-endothelial cell antibodies alone, and a fourth patient had mildly increased levels of antinuclear antibodies only. The clinical outcome was uneventful in all of the patients during an 18-month follow-up period. These findings suggest involvement of autoimmune factors associated with the coagulation system in some patients with idiopathic livedo reticularis, whose clinical significance remains to be determined.

Adolescent↗

Primary oxalosis with livedo reticularis.

Livedo reticularis developed on the lower extremities of a 37-year-old woman with primary oxalosis and renal failure that was treated by hemodialysis. Biopsy results of the involved skin indicated oxalate crystals in the walls of the blood vessels of the dermis and subcutaneous tissue. Oxalosis with vascular involvement should be included in the long list of causes of livedo reticularis.

Adult↗

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review.

Livedo is a cutaneous sign of striking violaceous netlike patterned erythema of the skin. This dermatological phenomenon is of special interest in the differential diagnosis in neurological patients. In 1907 Ehrmann distinguished two different patterns of livedo: the pathological livedo racemosa and the physiological livedo reticularis. Despite important clinical differences, in the English language literature the heading livedo reticularis is still used for all types of livedo. A literature review about the spectrum of differential diagnosis in patients with livedo reticularis (especially cutis marmorata and amantadine-induced livedo reticularis) and livedo racemosa (especially Sneddon's syndrome, Divry-van Bogaert syndrome, systemic lupus erythematosus, antiphospholipid antibody syndrome, polyarteritis nodosa, cholesterol embolization syndrome, livedoid vasculopathy and haematological diseases) is provided.

Adolescent↗

[Anti-beta 2 glycoprotein 1 antibodies in idiopathic livedo reticularis].

INTRODUCTION: Idiopathic livedo reticularis can be a sign of systemic disease since certain complications are frequently associated: cerebral thrombotic events in Sneddon's syndrome, necrotic ulcerations of the lower limbs. Antiphospholipid antibodies have been found in 0 to 85 p. 100 of patients with Sneddon's syndrome and anti-beta 2-glycoprotein 1 antibodies in 65 p. 100 of a series of 20 cases with Sneddon's syndrome. The aim of our study was to determine the prevalence of anti-beta 2-glycoprotein 1 antibodies in idiopathic livedo reticularis. PATIENTS AND METHODS: Twelve patients in a series of 17 with idiopathic livedo reticularis seen between 1981 and 1992 were studied progressively. All underwent a clinical examination and simple laboratory tests with search for anticardiolipin antibodies, lupus type circulating anticoagulant and anti-beta 2-glycoprotein 1 antibodies. RESULTS: Eight of our 12 patients (60 p. 100) were positive for anti-beta 2-glycoprotein 1 antibodies, 3 of whom also had episodes of thrombosis similar to those described in antiphospholipid syndrome. Only one of the 8 patients also had anticardiolipin antibodies and no chronic manifestation of thrombosis. DISCUSSION: beta 2-glycoprotein 1 is a cofactor which increases anticardiolipin antibody adhesion to cardiolipin in ELISA. Anti-beta 2-glycoprotein 1 antibodies are associated with thrombosis and antiphospholipid antibodies with lupus. Our results would suggest that the prevalence of anti-beta 2-glycoprotein 1 antibodies is high in idiopathic livedo, but, due to the small number of patients, do not allow confirmation that anti-beta 2-glycoprotein 1 antibodies are associated with thrombosis. Anti-beta 2-glycoprotein 1 antibody assay would be justified in routine evaluation of patients with livedo and at follow-up examinations.

Adult↗

Livedo reticularis and cerebrovascular accidents.

Livedo reticularis is a clinical pattern of cutaneous vascular involvement that can be seen in association with many diseases. The association of livedo reticularis and cerebrovascular accidents has been reported rarely in the past. We report on a 52-year-old woman with marked generalized livedo reticularis and cerebrovascular accidents who exhibited endarteritis obliterans without other pathologic changes on histologic examination of the skin. This constellation of findings suggest a primary vasculopathy with rather extensive involvement which merits our attention and further study.

Adult↗

Migraine is associated with livedo reticularis: a prospective study.

OBJECTIVE: To investigate the relationship of livedo reticularis, an ischemic dermatopathy, and migraine, an ischemic stroke risk factor. BACKGROUND: Livedo reticularis refers to the reddish-blue reticular mottling of the skin resulting from narrowing of small and medium arteries at the dermis-subcutis border. A subset of patients with livedo reticularis develop stroke in the absence of other vascular risk factors, which has been termed Sneddon syndrome. We undertook this prospective study in a non-neurology clinic to delineate further the relationship of livedo reticularis and migraine. METHODS: Patients in a general dermatology clinic were interviewed for vascular risk factors and history of migraine in accordance with the International Headache Society (IHS) criteria. A dermatologist, not familiar with the interview, recorded the primary dermatological diagnosis and the presence or absence of livedo reticularis on examination. RESULTS: Two hundred eighty-one consecutive patients (184 women and 97 men; average age, 52 years) were interviewed and examined. Seventy-five (27%) had migraine (IHS codes 1.1, 1.2) and an additional 18 (6%) had atypical migraine (IHS 1.7). Livedo reticularis was noted in 46 patients (16%), with the frequency higher in women than men (42 [23%] of 184 versus 4 [4%] of 97; P <.0001). The frequency of livedo reticularis in patients with migraine was higher than in those without migraine (24 [26%] of 93 versus 22 [12%] of 188; P =.002), and higher in female than male migraineurs (23 [32%] of 72 versus 1 [5%] of 21; P =.012). In logistic regression analysis of the women, migraine was associated with livedo reticularis (odds ratio [OR], 2.3; confidence interval [CI], 1.08 to 4.71), as well as with stroke (OR, 4.0; CI, 0.87 to 18.21), coronary artery disease (OR, 3.5; CI, 1.16 to 10.33), and deep venous thrombosis (OR, 3.2; CI, 0.98 to 10.32). CONCLUSIONS: In women, migraine is associated with stroke, coronary artery disease, deep venous thrombosis, as well as livedo reticularis, a dermatopathy which has been pathologically linked to cerebral vasculopathy. Whether migraineurs with livedo reticularis compose a subset at higher risk of thrombosis, including stroke, deserves further investigation.

Female↗

Livedo reticularis: an update.

Livedo reticularis (LR) is a well-known, relatively common physical finding consisting of macular, violaceous, connecting rings that form a netlike pattern (Fig 1). In most cases, it is a completely benign finding related to cold exposure. However, there are many potential causes (Table I), and this can make the evaluation of a patient presenting with this finding very difficult. An excellent review of the topic by Fleischer and Resnick was published in 1990. We have endeavored to update the literature and provide clinicians with guidance regarding the evaluation and treatment of patients presenting with LR.

Algorithms↗

Livedo reticularis and migraine: a marker for stroke risk?

BACKGROUND AND PURPOSE: Livedo reticularis is a dermatopathy characterized by an irregular, violaceous, netlike pattern which spares the face. Associated with a variety of conditions, it occurs consequent to pathological or physiological narrowing of small and medium arteries at the dermis-subcutis border. Sneddon syndrome refers to the idiopathic coupling of livedo reticularis and stroke in the absence of traditional vascular risk factors. Over 50% of persons with Sneddon syndrome describe a history of headache. We undertook this study to determine the frequency of livedo reticularis in our headache clinic. METHODS: We performed a retrospective chart review of consecutive patients attending a headache clinic over a period of 6 months. The patients had all been seen by one physician (G.E.T.) who noted the presence or absence of livedo reticularis. The charts were reviewed for age, sex, and vascular risk factors, including current use of oral contraceptives, and history of smoking, coronary artery disease, hypertension, diabetes mellitus, stroke, or arthritis. RESULTS: Charts from 133 patients with headache were reviewed (24 men [18%], 109 women [82%]; mean age, 42 +/- 13 years). Livedo reticularis was observed in 29 patients (22%) and in a similar proportion in men (25%) and women (21%). When we stratified the migraine population by presence or absence of livedo, we found no significant difference in age (44.5 versus 41.7 years, P =.16). There was a higher frequency of stroke diagnosis in the cohort with livedo reticularis (28%[8 of 29] versus 7%[7 of 104], P =.005), but we found no significant differences in frequency of hypertension, oral contraceptive use, diabetes mellitus, coronary artery disease, arthritis, or cigarette smoking. CONCLUSIONS: In our headache clinic, livedo reticularis is present in more than one fifth of patients. A history of stroke is more frequent in this subset of migraineurs, raising the possibility that livedo reticularis can be used as a clinical marker to identify those migraineurs with an increased risk of stroke.

Adult↗

[Vascular dementia and livedo reticularis].

A case of idiopathic livedo reticularis with dementia is described in a 37 year-old women. The symptoms of livedo reticularis preceded the onset of dementia by more than 16 years. Cerebral angiographic examinations gave evidence of cerebrovascular disease.

Adult↗

Anti-Pr cold hemagglutination associated with livedo reticularis.

A report of anti-Pr cold hemagglutination associated with livedo reticularis is presented. Livedo reticularis is descriptive of the superficial venous system appearing when blood flow is altered in this area. Cold antibody directed against the Pr antigen of the red cell membrane can cause hemagglutination, thereby altering blood flow in cooler areas of the body. This patient had livedo reticularis approximately 8 months prior to detection of the anti-Pr cold agglutinin.

Anemia, Hemolytic, Autoimmune↗

Livedo reticularis associated with increased titers of anticardiolipin antibodies in systemic lupus erythematosus.

Seventy-eight consecutive patients with systemic lupus erythematosus were assessed for the presence of livedo reticularis. The possible association of livedo reticularis with other clinical and laboratory features including anticardiolipin antibodies was explored. Thirty-eight patients had livedo reticularis. Four cases were severe, 11 moderate, and 23 mild. There was a statistically significant association between the combined moderate and severe livedo reticularis group and elevated levels of anticardiolipin antibodies. The recognized association of anticardiolipin antibodies with thrombotic events suggests a possible pathogenetic role. The presence or history of central nervous system disease, renal disease, vasculitis, or lupus inhibitor was significantly associated with the moderate and severe livedo reticularis group. Livedo reticularis may be a cutaneous marker for the later development of important systemic events in systemic lupus erythematosus.

Adolescent↗

Livedo reticularis during amantadine treatment.

Livedo reticularis is a common side effect of treatment with amantadine for Parkinson's disease. Investigation of 40 such patients suggests that the livedo is a physiological response provoked by depletion of catecholamine stores in peripheral nerve terminals.

Aged↗

Livedo reticularis.

A middle-aged woman was admitted to the hospital with livedo reticularis of the extremities in addition to nonspecific symptoms and signs of hypothyroidism. The livedo reticularis disappeared with treatment of the hypothyroidism. Although livedo reticularis has been reported previously to be associated with thyroid disease, this association is rare. We review the literature and present the conditions associated with livedo reticularis.

Adult↗

Livedo reticularis and livedoid vasculitis responding to PUVA therapy.

Livedo reticularis is a mottled blue discoloration of the skin, which occurs in a netlike pattern. Livedoid vasculitis is a chronic disorder clinically manifested by recurrent painful ulcerations of the lower extremities and is characterized by the presence of smooth or depressed ivory-white lesions surrounded by hyperpigmentation and telangiectasia. We describe two patients with livedo reticularis and livedoid vasculitis who responded to PUVA therapy and propose that systemic PUVA with methoxsalen undergo further investigation as an alternative therapy for drug-resistant patients with livedo reticularis and livedoid vasculitis.

Adult↗

Livedo reticularis: a rare manifestation of Graves hyperthyroidism associated with anticardiolipin antibodies.

Livedo reticularis is a common presentation of the anticardiolipin syndrome. Although the presence of anticardiolipin (aCL) antibodies was previously reported in association with both Graves disease and Hashimoto thyroiditis, I am aware of no previous report of livedo reticularis in patients with Graves disease. A 29-year-old woman presented with very active Graves thyrotoxicosis. Physical examination revealed, in addition to signs of very active hyperthyroidism, marked livedo reticularis on both her legs. Laboratory tests confirmed the clinical impression regarding her thyroid activity and revealed prolonged prothrombin time (and partial thromboplastin time) and elevated levels of both immunoglobulin G and immunoglobulin M aCL antibodies. After methimazole-induced remission, livedo reticularis completely disappeared and the levels of aCL antibodies decreased to within the low range of normal. The literature on aCL antibodies and autoimmune thyroid diseases is reviewed and the interaction between the occurrence of aCL antibodies, disease activity, and drug therapy in Graves disease is discussed.

Adult↗

Livedo reticularis: an underutilized diagnostic clue in cholesterol embolization syndrome.

BACKGROUND: Cholesterol embolization syndrome (CES) is an increasingly recognized cause of acute renal insufficiency, which must be differentiated from other forms of systemic vasculitis by histologic examination of biopsies from involved organs. This report describes the optimal methods for detection and biopsy of areas of skin involved with livedo reticularis to confirm the diagnosis of CES. METHODS: This report describes 8 patients with unexplained acute renal insufficiency in whom the diagnosis of CES was suspected based on their clinical history. RESULTS: A detailed skin examination performed in both supine and upright postures demonstrated the presence of previously unrecognized livedo reticularis, which was more evident during upright posture in all subjects. In 2 subjects, questionable areas of livedo reticularis noted in supine posture became readily demonstrable during upright posture. Livedo reticularis was apparent only during upright posture in 2 subjects. Biopsies of areas of skin involved with livedo reticularis demonstrated cholesterol emboli in 6 of 8 patients and were normal in the remaining 2 patients. One patient progressed to end-stage renal disease and one was lost to follow-up. In the remaining 6 patients, renal insufficiency initially progressed but did not require dialytic therapy. Renal function returned to baseline levels and livedo reticularis resolved without recurrence in these patients. No subjects developed clinical or laboratory evidence of systemic vasculitis. CONCLUSIONS: Livedo reticularis is a common but often unrecognized finding in CES that may not be evident during routine examination performed in the supine posture. Deep cutaneous biopsy of areas of livedo reticularis can be safely used to confirm the presence of cholesterol emboli, thus avoiding the increased morbidity of biopsy of either pregangrenous skin lesions or visceral organs. Many patients with CES regain renal function during long-term follow-up.

Aged↗