Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Livedoid vasculitis, a hyalinizing vasculopathy, is characterized by extensive formation of microthrombi and deposition of fibrin in the middermal vessels, which result in epidermal infarction, ulceration, and formation of stellate scars. In a prospective study of nonhealing ulcers in patients with livedoid vasculitis, we found a high incidence of anticardiolipin antibodies, lupus anticoagulants, increased levels of plasminogen activator inhibitor, and low levels of endogenous tissue plasminogen activator (t-PA) activity. This procoagulant tendency and decreased fibrinolysis may provide an explanation for the occlusive vasculopathy often noted in biopsy specimens from these patients. On the basis of these findings, we proposed that fibrinolysis with recombinant t-PA would lyse microvascular thrombi, restore circulation, and promote wound healing. In six patients who had nonhealing ulcers caused by livedoid vasculitis and in whom numerous conventional therapies had failed, low-dose t-PA (10 mg) was administered intravenously during a 4-hour period daily for 14 days. Five of the six patients had dramatic improvement; almost complete healing of the ulcers occurred during hospitalization, and tissue oxygenation, as measured by transcutaneous oximetry, increased. The one treatment failure was due to rethrombosis of the microvasculature; this patient was subsequently re-treated but with concurrent anticoagulation, and her leg ulcers healed. We conclude that daily administration of a low dose of t-PA is safe and effective treatment for nonhealing ulcers due to occlusive vasculopathy.
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PMID:Tissue plasminogen activator for treatment of livedoid vasculitis. 143 47

Of 66 patients with systemic lupus erythematosus (SLE), 11 (16.7%) had livedoid vasculitis. The sites of predilection included the elbows, knees, fingers, soles, and back, which are not those of common livedoid vasculitis. Livedoid vasculitis developed in ten (90.9%) of the 11 patients with SLE who eventually developed central nervous system (CNS) lupus. In contrast, such preceding livedoid vasculitis occurred in only three (11.5%) of the 26 patients with severe nephrotic lupus nephritis. Livedoid vasculitis did not occur in any patient with neither CNS lupus nor severe lupus nephritis. These results indicate that livedoid vasculitis in SLE is a very important cutaneous manifestation that may possibly be prodromal to CNS lupus.
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PMID:Livedoid vasculitis and central nervous system involvement in systemic lupus erythematosus. 394 9

Seven patients with scleroderma and either livedo reticularis or atrophie blanche lesions had ulcers of the lower extremity. Livedoid vasculitis, periarteritis nodosa, or endarteritis obliterans lesions were associated with the ulcers. Five patients had livedoid vasculitis, one patient had associated lupus panniculitis, and one patient had rheumatoid arthritis. Four patients had elevated ESRs, four had positive antinuclear antibody tests, and two had positive tests for rheumatoid factor. Patients with scleroderma and livedoid vasculitis or livedo reticularis and ulcers should be examined to rule out underlying vascular disease or endarteritis obliterans.
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PMID:Vascular ulcers in scleroderma. 613 96

Many pathophysiological process components are known to be implicated in lower limb ulcerations, among which vascular lesions have a major role. Vasculitis denotes a heterogeneous group of clinical entities which all are characterized by the inflammatory process of arterial and venous walls of any size and in any organ, quite frequently in the skin. Vasculopathy, on the other hand, refers to vascular and capillary lesions caused by, for example, some medications. The classification of vasculitides according to the size of the blood vessels involved serves for proper understanding the issue among clinicians and researchers, and not as a diagnostic tool. According to histologic finding obtained by examination of blood vessel biopsy specimen, vasculitides are divided into three groups: lymphocytic, leukocytoclastic and granulomatous. Livedoid vasculitis (livedo reticularis) most commonly affects women and is generally localized on lower extremities. The etiology oflivedoid vasculitis may imply autoimmune diseases, capillary obstruction with cryoglobulins, or antiphospholipid syndrome. Livedoid vasculopathy is a hyalinization disease of the vasculature, with thromboses and ulcerations on lower extremities, and of unknown etiology. Livedoid vasculopathy has been singled out as a separate disease that usually does not occur consequentially to other primary diseases. Livedoid vasculopathy typically affects women (71%) at a mean age of 45 (range 10-85) years; bilateral involvement of both lower limbs is present in 80.8%, disease manifested with ulcerations in 68.9%, ulcerations followed by development of atrophie blanche in 71.1%, transcutaneous oximetry reduction is found in 74.1%, factor V mutation (Leiden heterozygotes) in 22.2%, reduced protein C activity in 13.3%, prothrombin gene mutation (G20210A) in 8.3%, positive lupus anticoagulant in 17.9%, positive anticardiolipin antibodies in 28.6%, and elevated homocysteine level in 14.3% cases; blood vessel histology shows intraluminal thrombosis in 97.8% of patients, while direct immunofluorescence of blood vessel specimen shows immunoglobulins and complement components in blood vessels on the surface, in the mid-dermis as well as deep in the dermis. The immunofluorescence pattern differs from that found in immune complex diseases. Some of the agents tried in the treatment of livedoid vasculopathy include pentoxifylline, low-molecular heparin, hyperbaric oxygen therapy, methylprednisolone i.v. with pentoxifylline, recombinant tissue plasminogen activator, intravenous immunoglobulins, phenformin (biguanide) and ethylestrenol (anabolic steroid) combination, warfarin, heparin, systemic photochemotherapy (PUVA with oral psoralen), and low-molecular dextran. Infected ulcerations are treated with antibiotics. Combined therapy with folic acid, vitamin B12 and vitamin B6 can also be used.
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PMID:[Vasculitis and vasculopathy]. 2319 16