Mixed Connective Tissue Disease Associated with Skin Defects of Livedoid Vasculitis

Oh, Yoo-Jeong; Jun, Jae‐Bum; Kim, C. K.; Lee, C. W.; Park, C. K.; Kim, T.-Y.; Yoo, Dae Hyun; Kim, S. Y.

doi:10.1007/s100670070032

Cited by 21 publications

(13 citation statements)

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“…The disease is considered to be an occlusive thrombotic process due to a potential defect in the hemostasis. There have been no reports of organ involvement with LV, although the disease has been associated with signs of peripheral venous insufficiency and collagen vascular disease [19]. The histopathology findings support this observation as the lesions characteristically include segmental hyalinization, endothelial proliferation, and thrombosis of the upper and mid-dermal blood vessels [6].…”

Section: Discussionmentioning

confidence: 83%

Enhanced functional stability of plasminogen activator inhibitor-1 in patients with livedoid vasculopathy

Ağırbaşlı

Eren

et al. 2011

J Thromb Thrombolysis

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Livedoid vasculopathy (LV) is a chronic, recurrent, painful cutaneous disease with distinctive clinical features and an uncertain etiology. The skin lesions are recognizable by focal purpura, depigmentation and shallow ulcers. Thrombophilic conditions occur frequently in patients with LV. While no definitive treatment exists for LV, smoking cessation, antiplatelet therapy, immunosuppressive treatment, and anabolic steroids are often included in the therapeutic ladder. Recently, a possible link between LV and impaired fibrinolysis was established as cutaneous LV lesions responded to tissue plasminogen activator (t-PA) infusion suggesting that inhibition of the fibrinolysis through plasminogen activator inhibitor-1 (PAI-1) activity may determine the disease course in patients with LV. In this study, we investigated PAI-1 antigen (Ag) and activity levels in 20 patients with biopsy proven LV (mean age 26 ± 11, M/F = 7/13, median disease duration 3.5 years). All patients received antiplatelet treatment with aspirin and/or dipyrimadole and 14 patients received anabolic steroids or immunosuppressive treatment. Fasting PAI-1 Ag and activity levels were measured at 9 AM in all patients. Both Ag (34 (26) ng/ml) (median (interquartile range)) and specific activity (17 (23) IU/fmole) levels of PAI-1 were moderately elevated in LV patients compared to the controls, however, PAI-1 kinetic studies demonstrated markedly enhanced stability of PAI-1 activity in plasma from patients with LV. Specific activity at 16 h was significantly higher than expected specific activity levels (7 (11) vs. 0.07 (0.09) IU/fmole, P < 0.01). While the exact mechanism of increased stability of PAI-1 activity is not known, it may be due to post-translational modifications or increased binding affinity for a stabilizing cofactor. In conclusion, enhanced stability of PAI-1 may contribute to the pathophysiology of LV, and systemic or local treatment with PAI-1 inhibitors may offer a potential treatment alternative in patients with LV.

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Section: Discussionmentioning

confidence: 83%

Enhanced functional stability of plasminogen activator inhibitor-1 in patients with livedoid vasculopathy

Ağırbaşlı

Eren

et al. 2011

J Thromb Thrombolysis

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“…Disease mainly affects dermal layers, yet there have been no reports of peripheral venous insufficiency and collagen vascular diseases associated with LV . The common hypothesis is that LV is a primary thrombotic disturbance at the microcirculatory level, determining a local state of hypercoagulability, instead of being a vasculitic process .…”

Section: Discussionmentioning

confidence: 99%

Enhanced mRNA expression of plasminogen activator inhibitor‐1 in livedoid vasculopathy lesions

Ağırbaşlı

Göktay

Peker

et al. 2017

Cardiovascular Therapeutics

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“…[11] The classical puffy finger of MCTD does not show features of sclerodactyly. [14] The clinical and serological markers of MCTD take time to develop. [111516] Period of illness among those with MCTD in our group ranged from 7 months to 120 months.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous manifestations of mixed connective tissue disease: Study from a tertiary care hospital in Eastern India

et al. 2014

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Context:Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe.Aims:This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe.Settings and Design:Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years.Materials and Methods:The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients.Statistical Analysis Used:SPSS (Version 17) and MedCalc (Version 11.6).Results:The Male: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynaud's phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this diseaseConclusions:Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease.

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Mixed Connective Tissue Disease Associated with Skin Defects of Livedoid Vasculitis

Cited by 21 publications

References 0 publications

Enhanced functional stability of plasminogen activator inhibitor-1 in patients with livedoid vasculopathy

Enhanced functional stability of plasminogen activator inhibitor-1 in patients with livedoid vasculopathy

Enhanced mRNA expression of plasminogen activator inhibitor‐1 in livedoid vasculopathy lesions

Cutaneous manifestations of mixed connective tissue disease: Study from a tertiary care hospital in Eastern India

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