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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↗

Heparin-associated thrombocytopenia and thrombosis (HATT) presenting with livedo reticularis.

BACKGROUND: Heparin-associated thrombocytopenia and thrombosis (HATT) is an infrequently encountered syndrome characterized by ischemic necrosis of soft tissue and vital organs following anticoagulation with heparin. The syndrome is thought to be due to heparin-dependent platelet aggregation and thrombosis, which is mediated by pathologic immunoglobulins. CASE REPORT: A 60-year-old man developed truncal livedo reticularis and ischemic necrosis of the left foot associated with thrombocytopenia, disseminated intravascular coagulopathy (DIC), and microangiopathic hemolytic anemia during intravenous heparin therapy. Skin biopsy from an area of livedo reticularis revealed fibrin thrombi in dermal blood vessels, which is characteristic of HATT. The diagnosis of HATT promoted discontinuation of heparin and a resulting rapid resolution of the livedo reticularis and hematologic abnormalities. No other potential causes of DIC were identified, and, other than stopping heparin, no specific therapy was employed. CONCLUSIONS: Periodic monitoring of platelets should be performed on all patients receiving treatment with heparin, as early detection of heparin-induced thrombocytopenia followed by discontinuation of the drug may prevent life threatening thrombotic complications. HATT should be included in the differential diagnosis of patients with livedo reticularis that occurs during heparin therapy.

Biopsy↗

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, developmental delay and stroke-like episode in a 7-year-old male.

Livedo reticularis is a vascular abnormality of the skin resulting in an erythematous reticular rash. The combination of livedo reticularis and stroke-like episodes in adults is known as Sneddon syndrome [Sneddon, IB (1965). Br J Dermatol 77:180-188]. A similar combination of stroke-like episodes and livedo reticularis has been reported to occur in children [Baxter P et al. (1993). Dev Med Child Neuro 35:917-926]. We present here a 7-year-old male with congenital livedo reticularis, obesity, developmental delay, stroke-like episode, hypertension and cystic kidneys. We summarize our patient's findings and family history, and compare his disorder to other possibly related conditions.

Adult↗

[A case of livedo vasculitis associated with mononeuritis multiplex].

Livedo vasculitis is characterized by recurrent livedo reticularis of lower extremities and the histopathological findings of segmental hyalinizing vasculitis in the skin. We report a case of a 26-year-old female who manifested mononeuritis multiplex 7 years after the onset of livedo vasculitis. She showed sensori-motor disturbances in the right median and ulnar nerves and sensory deficits of the bilateral peroneal nerves. Sural nerve biopsy revealed a remarkable loss of large and small myelinated fibers and a few vasculitic changes. Steroids therapy was effective for these neurological symptoms. But paroxysmal numbness appeared later recurrently in the regions of affected nerves with painful ulcerations in the right leg. Laboratory tests indicated increased levels of serum thrombin-antithrombin complex (TAT), and antithrombotic drugs (argatroban) remarkably ameliorated the recurrent symptoms and skin lesions. These findings suggest that the pathogenesis of livedo vasculitis might be related to alterations of the blood coagulation system.

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 racemosa generalisata: an evaluation of thirty-four cases.

The results of investigations in 34 patients (28 women, 6 men) with livedo racemosa generalisata are presented. Neurologic or psychiatric symptoms were present in 28 patients. Nineteen patients had had one or more cerebral infarctions, and epilepsy (Sneddon's syndrome) developed in six. In most cases livedo preceded the neurologic disorder. In addition, many patients with livedo racemosa generalisata had Raynaud's phenomenon, cardiac abnormalities, or vascular changes in the ocular fundus.

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↗

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↗

[Critical ischaemia of the limbs and localized livedo in a case of ergotism].

HISTORY AND ADMISSION FINDINGS: A 57-year-old woman, a heavy smoker and migraine sufferer, was admitted with severe resting pain in the right forefoot and painful localized tendril-shaped reddening on the right thigh. She had regularly been taking 1-2 mg ergotamine tartrate, several analgesics, some containing caffeine, and selective serotonin-uptake inhibitors. Clinical examination found all limbs to be cool. On the right leg the pulse was not palpable below the inguinal line, and the reddening corresponded to localized livedo. INVESTIGATIONS: The peripheral Doppler pressure indicated critical perfusion reduction in the right leg with a tibiobrachial pressure ratio of 0.14. Colour-coded duplex sonography showed generalized vasoconstriction with filiform hourglass stenosis of the right proximal superficial femoral artery without atherosclerotic changes. The history of drug intake and the characteristic sonographic findings indicated ergotism and an arteriography was deemed unnecessary. TREATMENT AND COURSE: All ergotamine and caffeine containing drugs were discontinued and the patient urged to stop smoking. Amlidopine, 2.5 mg orally, and prostaglandin E1, 60 microgram i.v., were administered daily. The resting pain was much reduced after the first infusion and the painful livedo disappeared. The documented high-grade stenosis of the right superficial femoral artery was reduced to 25-50% by the third day of infusion. At the end of 10 daily infusions both the Doppler pressure and the duplex sonography had become normal. Pizotifen was given for the migraine and the serotonin re-uptake inhibitor sertralin was discontinued. CONCLUSION: An interaction of the serotonin re-uptake inhibitor with ergotamine was presumably responsible for the development of ergotism under >>therapeutic<< ergotamine dosage. Vasospastic stenoses and occlusions can be demonstrated by duplex sonography and may in future not require additional angiographic confirmation. Intravenous rather than intraarterial infusion of prostaglandin is to be preferred if vessels at many sites are affected. Livedo is a transitory sign of ergotism.

Arm↗

Reticulate purpura, cryoglobulinaemia and livedo reticularis.

Two patients with type I cryoglobulinaemia are described, both of whom presented with purpura in a reticulate distribution on the legs, resembling the pattern of physiological livedo. It appeared that increased cooling due to sluggish blood flow in areas of the skin corresponding to the blue areas of physiological livedo may have caused the localization of cryoprecipitate at these sites. To investigate the pathogenesis of the net-like pattern of purpura, three subjects with physiological livedo reticularis of the thighs were studied with a laser-Doppler velocimeter. In two subjects, mean blood flux in the blue areas was 21% (P < 0.01) and 26% (P < 0.02) lower than in adjacent white areas, whereas in the third there was no significant difference. The cutaneous features of cryoglobulinaemia, and the mechanism of the reticulate purpura in this condition, are discussed.

Aged↗

Livedo reticularis associated with hereditary protein C deficiency and recurrent thromboembolism.

We report the occurrence of livedo reticularis in a patient with symptomatic hereditary type 1 protein C deficiency. Antithrombin III deficiency and the antiphospholipid syndrome may also be associated with livedo reticularis, and we suggest that a thrombophilia screen may be a useful investigation in a patient with otherwise unexplained livedo, particularly if there is a personal or family history of thromboembolism.

Adult↗

Yq- in a child with livedo reticularis, snub nose, microcephaly, and profound mental retardation.

A child with terminal deletion of the long arm of the Y chromosome (Yq--) presented with marked livedo reticularis, snub nose, microcephaly, short stature, and other dysmorphic features. He was profoundly mentally retarded. Most of the patients with Yq- have been reported as having varying dysmorphic features, mental retardation, and short stature. This child, in addition to the above, has livedo reticularis and microcephaly. He was of normal birthweight and, therefore, does not come into the syndrome of microcephaly, snub nose, livedo reticularis, and low birthweight dwarfism. Further information on Yq- should be obtained to ascertain if consistent patterns of abnormalities exist.

Body Height↗

Livedo reticularis is a marker for predicting multi-system thrombosis in antiphospholipid syndrome.

OBJECTIVE: Livedo reticularis (LR) is a skin vasculopathy that has been frequently described in patients with anti-phospholipid syndrome (APS) and reported to be present in association with valvular heart pathology and strokes (i.e. Sneddon's syndrome). METHODS: In a cohort of APS patients we investigated the possible association of LR with various clinical aspects of APS such as pregnancy morbidity, central nervous system (CNS) and cardiac manifestations. RESULTS: Livedo reticularis was found in 50/308 (16%) of APS patients, and there was a significant association with cerebrovascular accidents (CVA), migraines and epilepsy (p = 0.01, 0.002, and 0.02 respectively). A similar association was also detected between LR, and the presence of cardiac valve thickening and vegetations (p = 0.001). No association with venous thrombosis, recurrent fetal loss, IUGR or toxemia was found. CONCLUSION: Livedo reticularis is a frequent cutaneous manifestation in patients with APS. Its high association with cardiac and CNS thrombosis may suggest that LR-APS patients compose a subset at higher risk for thrombosis, and thus may require a closer follow-up and a more aggressive anticoagulation.

Adult↗

[Livedo racemosa].

Livedo racemosa is a very rare disease with an idiopathic and symptomatic form. The diagnosis is very difficult to make because many primary diseases are responsible. Skin reactions show no typical form and range from urticarial nodules and purpuriform papillae to ulceration. The symptomatic form of livedo racemosa causes circumscribed, asymmetric lesions restricted to one half of the body, while the idiopathic form is characterised by arborization figures and livid tendril-like discolorations. There are many suggestions given in the literature for the therapy of the primary disease and of the skin, including long term therapy with high dosed rubifacient drugs or steroids. However, livedo racemosa often shows a slow progression despite treatment.

Adult↗

[Cerebrovascular disorders in young patients with livedo racemosa generalisata (Ehrmann)].

The results of examinations on 15 patients suffering from livedo racemosa generalisata (idiopathic livedo reticularis) are described. 12 of the patients were found to have cerebrovascular disturbances (in most cases severe) and 2 of them had migraine accompagnée. The remaining patient had no neurological changes. The majority of the patients were women (12 female, 3 male). In most cases the illness began between the age of 20 and 30 years. Cerebral angiographic examinations showed the existence of peripheral angiostenosis, whereas in 9 patients pronounced cases of diffused cerebral atrophy could be found with the help of computerised tomography. Questions concerning the nosological position, pathogenesis and therapy of livedo racemosa generalisata are discussed.

Adult↗

Position dependent livedo reticularis in cholesterol emboli syndrome.

We describe a case of a 64-year-old Filipino man who presented with cholesterol emboli syndrome manifesting as worsening hypertension, renal failure and livedo reticularis involving the upper legs and lower abdomen. The livedo reticularis became very prominent with the patient standing, but completely vanished after several minutes of lying supine. Deep cutaneous biopsy of an area of skin that was found to be consistently involved with livedo reticularis demonstrated cholesterol clefts in several vessels, thus establishing the diagnosis in this patient, and avoiding the more problematic option of biopsying an involved visceral organ.

Biopsy↗

[Livedo vasculitis: report of a case].

Livedo vasculitis is an occlusive thrombotic hyalinizing vessel disease characterized by parietal hyalinization, endothelial proliferation, fibrin deposits and formation of thrombi within the superficial and deep dermal vessels. Diagnosis, essentially clinical, emphasizes the clinical and histopathological features of livedo vasculitis. We conducted an etiological investigation in our case, as required to diagnose idiopathic livedo vasculitis. Several therapies have been suggested with inconstant results and recurrence after withdrawal. In our case, treatment with recombinant tissue plasminogen activator was successful but was unable to prevent recurrence.

Adult↗