Behçet's disease with severe cutaneous necrotizing vasculitis: Response to plasma exchange—Report of a case

Cornélis, François; Sigal-Nahum, M; Gaulier, A; Bleichner, G; Sigal, S

doi:10.1016/s0190-9622(89)80234-8

Cited by 21 publications

(10 citation statements)

References 13 publications

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“…Yet, in the present study, many male patients were positive for this antibody, and there was no history of transfusion or pregnancy among any of the antibody-positive BD patients. Previous studies have shown higher IgM positivity compared with IgG positivity for AECA in BD (20), and direct immunofluorescence has shown a predominance of IgM class among the immunoglobulins deposited in the venular walls of BD lesions (19,37). The present study also showed that the reactivity to HDMEC ␣-enolase was detected only in IgM-positive BD sera.…”

Section: Aeca Against ␣-Enolase In Behç Et's Disease 2031supporting

confidence: 73%

“…In BD, vasculitis mainly involves capillaries and small vessels (2,3). Direct immunofluorescence of the lesions of BD patients mainly shows deposition of immunoglobulin on the walls of small veins (19). One study reported a higher prevalence of AECA when sera from BD patients were tested against microvascular endothelial cells as compared with human umbilical vein endothelial cells as the substrate (8).…”

Section: Aeca Against ␣-Enolase In Behç Et's Disease 2031mentioning

confidence: 99%

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Human α‐enolase from endothelial cells as a target antigen of anti–endothelial cell antibody in Behçet's disease

Lee¹,

Chung²,

Kim³

et al. 2003

Arthritis & Rheumatism

175

108

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Objective. To identify and recombine a protein of the human dermal microvascular endothelial cell (HD-MEC) that specifically reacts with anti-endothelial cell antibody (AECA) in the serum of patients with Behçet's disease (BD), and to evaluate the usefulness of this protein in BD.Methods. The proteomics technique, with 2-dimensional gel electrophoresis and matrix-assisted laser desorption ionization؊time-of-flight (MALDI-TOF) mass spectrometry, was used to identify and recombine HDMEC antigen. Western blotting and enzyme-linked immunosorbent assay (ELISA) of recombinant protein isolated by gene cloning were performed on serum from healthy controls, patients with BD, and patients with other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, and Wegener's granulomatosis).Results. Eighteen of 40 BD patients had serum IgM antibody to HDMEC antigen. The purified protein that reacted with AECA in BD patient sera was found to be ␣-enolase by 2-dimensional gel electrophoresis followed by immunoblotting and MALDI-TOF mass spectrometry. Recombinant ␣-enolase protein was isolated and refined by gene cloning. On Western blots, AECApositive IgM from the sera of patients with active BD reacted strongly with recombinant human ␣-enolase. BD patient sera positive for anti-␣-enolase did not react with human ␥-enolase. On dot-blotting, reactivity to human ␣-enolase was detected only in the IgM-positive group. Fifteen of the 18 AECA-positive sera that were positive for the HDMEC antigen showed reactivity to recombinant ␣-enolase IgM antibody by ELISA.Conclusion. The ␣-enolase protein is the target protein of serum AECA in BD patients. This is the first report of the presence of IgM antibodies to ␣-enolase in endothelial cells from the serum of BD patients. Although further studies relating this protein to the pathogenesis of BD will be necessary, ␣-enolase and its antibody may prove useful in the development of new diagnostic and treatment modalities in BD.

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Section: Aeca Against ␣-Enolase In Behç Et's Disease 2031supporting

confidence: 73%

Section: Aeca Against ␣-Enolase In Behç Et's Disease 2031mentioning

confidence: 99%

Human α‐enolase from endothelial cells as a target antigen of anti–endothelial cell antibody in Behçet's disease

Lee¹,

Chung²,

Kim³

et al. 2003

Arthritis & Rheumatism

175

108

View full text Add to dashboard Cite

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“…Other cutaneous vasculitic lesions include Sweet's syndrome-like,47,48 pyoderma gangrenosum-like,49 erythema multiforme-like lesions,50 palpable purpura,51 subungual infarctions, hemorrhagic bullae,52 furuncles, and abscesses 24…”

Section: Other Cutaneous Vasculitic Lesionsmentioning

confidence: 99%

Mucocutaneous Lesions of Behçet's Disease

2007

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Behçet's disease is particularly prevalent in "Silk Route" populations, but it has a global distribution. The diagnosis of the disease is based on clinical criteria as there is as yet no pathognomonic test, and mucocutaneous lesions, which figure prominently in the presentation and diagnosis, may be considered the diagnostic hallmarks. Among the internationally accepted criteria, painful oral and genital ulcers, cutaneous vasculitic lesions and reactivity of the skin to needle prick or injection (the pathergy reaction) are considered hallmarks of Behçet's disease, and often precede other manifestations. Their recognition may permit earlier diagnosis and treatment, with salutary results. This paper describes the various lesions that constitute the syndrome and focuses on those that may be considered characteristic.

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“…Most affected are patella, ankle, elbow and wrist. Bone change in joints is rare (6)(7)(8)(9)(10)(11).…”

mentioning

confidence: 99%

Are erythema nodosum-like lesions and superficial thrombophlebitis prodromal in terms of visceral involvement in Behcet's disease?

Coşkun

Öztürk

Saral

2005

International Journal of Clinical Practice

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Erythema nodosum (EN)-like lesions and superficial thrombophlebitis (ST) are skin symptoms that are frequently observed in Behcet's disease. In most of the patients, skin symptoms precede serious visceral involvement. The study included 78 patients whose mean age was 33.48 +/- 6.16 (21-48). Of the cases, 37 were females and 41 were males. Forty-two patients (53.8%) had EN-like lesion and 12 (15.3%) had ST. Six of 54 patients, who had EN-like lesion and ST, were found to have no visceral involvement. In six of the remaining 48 patients, it was established that visceral involvement developed before lesions. It was found in 42 patients that these two lesions preceded visceral involvement. In addition, 18 of 24 patients who did not have these two lesions, do not still have visceral involvement. Therefore, we think that EN-like lesion and ST can be guiding in predicting serious visceral involvement and complications that can develop in advanced stages of Behcet's disease.

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Behçet's disease with severe cutaneous necrotizing vasculitis: Response to plasma exchange—Report of a case

Cited by 21 publications

References 13 publications

Human α‐enolase from endothelial cells as a target antigen of anti–endothelial cell antibody in Behçet's disease

Human α‐enolase from endothelial cells as a target antigen of anti–endothelial cell antibody in Behçet's disease

Mucocutaneous Lesions of Behçet's Disease

Are erythema nodosum-like lesions and superficial thrombophlebitis prodromal in terms of visceral involvement in Behcet's disease?

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