Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involvemuscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of...Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involvemuscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. Onset usually occurs before the age of 14 years. We describe adultonset disabling pansclerotic morphea in two previously healthy young men. In both cases, the onset of disease was explosive, with rapid progression,widespread cutaneous involvement, and severe disablement caused by mutilating contracture deformities. Increased susceptibility of sclerodermatous tissue to recalcitrant ulceration and malignant transformation with development of nonmelanoma skin cancers was also observed. Treatment of this disease continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy.展开更多
Background: Anti-agalactosyl immunoglobulin G (IgG) antibodies (anti-AG IgG) have been reported to be detected and correlated with disease activity in some collagen diseases. Method: Forty-seven serum samples from pat...Background: Anti-agalactosyl immunoglobulin G (IgG) antibodies (anti-AG IgG) have been reported to be detected and correlated with disease activity in some collagen diseases. Method: Forty-seven serum samples from patients with localiz ed scleroderma were examined using an enzyme-linked immunosorbent assay. Result s: Anti-AG IgG were positive in 19%of patients with localized scleroderma. The frequency of anti-AG IgG in generalized morphea was much higher than that in l inear scleroderma or that in morphea.There was a significant correlation between anti-AG IgG levels and the number of the sclerotic lesions and between anti-A G IgG levels and the number of involved areas. The levels of anti-AG IgG were s ignificantly higher in patients with antinuclear antibody, antisingle-stranded DNA antibody or rheumatoid factor than in those without. Conclusion: Anti-AG Ig G can be an indicator of the severity of localized scleroderma.展开更多
Background:Localized scleroderma (LSc) exhibits autoimmunity, and antihistone antibody is frequently detected. The major antigens recognized by antihistone a ntibody are histones H1, H2AandH2B,whicharelocatedontheoute...Background:Localized scleroderma (LSc) exhibits autoimmunity, and antihistone antibody is frequently detected. The major antigens recognized by antihistone a ntibody are histones H1, H2AandH2B,whicharelocatedontheoutersideofthenucleosome and are relatively more accessible for antibody binding. Therefore, it has been hypothesized that antihistone antibody is induced by nucleosome or native chroma tin as immunogens in LSc. Objectives:To determine whether antinucleosome antibo dy is present in patients with LSc. Methods:Antinucleosome antibody, antihiston e antibody and antidouble-stranded DNA (dsDNA) antibody were determined by enzy me-linked immunosorbent assay. Results:IgG or IgM antinucleosome antibody was detected more frequently in patients with LSc than was antihistone antibody:in 40 of 49 (82%) vs. 26 of 49 (53%), respectively. No patients had anti-dsDNA a ntibody. The prevalence of antinucleosome antibody positivity was comparable in the three subgroups of LSc (generalized morphoea, 89%; linear scleroderma, 71% ; morphoea, 90%). Patients with systemic lupus erythematosus (SLE) exhibited a similar frequency of antinucleosome antibody positivity (13 of 15, 87%), but th eir IgG levels of this autoantibody were much higher than those found in patient s with LSc. By contrast, IgM antinucleosome antibody levels were normal in patie nts with SLE, while they were significantly increased in patients with LSc compa red with normal controls. Antinucleosome antibody was also detected at lower fre quency in patients with systemic sclerosis(fiveof20,25%)ordermatomyositis(fiveo f15,33%). Nucleosome-restricted antibodies, i.e. antibodies that react with th e whole nucleosome particle but not with its individual components (histones and dsDNA) were also present in 35%of patients with LSc. Conclusions:Although ant inucleosome antibody was not specific to LSc, its high prevalence in LSc indicat es that antinucleosome antibody is a major autoantibody in this disease.展开更多
文摘Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involvemuscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. Onset usually occurs before the age of 14 years. We describe adultonset disabling pansclerotic morphea in two previously healthy young men. In both cases, the onset of disease was explosive, with rapid progression,widespread cutaneous involvement, and severe disablement caused by mutilating contracture deformities. Increased susceptibility of sclerodermatous tissue to recalcitrant ulceration and malignant transformation with development of nonmelanoma skin cancers was also observed. Treatment of this disease continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy.
文摘Background: Anti-agalactosyl immunoglobulin G (IgG) antibodies (anti-AG IgG) have been reported to be detected and correlated with disease activity in some collagen diseases. Method: Forty-seven serum samples from patients with localiz ed scleroderma were examined using an enzyme-linked immunosorbent assay. Result s: Anti-AG IgG were positive in 19%of patients with localized scleroderma. The frequency of anti-AG IgG in generalized morphea was much higher than that in l inear scleroderma or that in morphea.There was a significant correlation between anti-AG IgG levels and the number of the sclerotic lesions and between anti-A G IgG levels and the number of involved areas. The levels of anti-AG IgG were s ignificantly higher in patients with antinuclear antibody, antisingle-stranded DNA antibody or rheumatoid factor than in those without. Conclusion: Anti-AG Ig G can be an indicator of the severity of localized scleroderma.
文摘Background:Localized scleroderma (LSc) exhibits autoimmunity, and antihistone antibody is frequently detected. The major antigens recognized by antihistone a ntibody are histones H1, H2AandH2B,whicharelocatedontheoutersideofthenucleosome and are relatively more accessible for antibody binding. Therefore, it has been hypothesized that antihistone antibody is induced by nucleosome or native chroma tin as immunogens in LSc. Objectives:To determine whether antinucleosome antibo dy is present in patients with LSc. Methods:Antinucleosome antibody, antihiston e antibody and antidouble-stranded DNA (dsDNA) antibody were determined by enzy me-linked immunosorbent assay. Results:IgG or IgM antinucleosome antibody was detected more frequently in patients with LSc than was antihistone antibody:in 40 of 49 (82%) vs. 26 of 49 (53%), respectively. No patients had anti-dsDNA a ntibody. The prevalence of antinucleosome antibody positivity was comparable in the three subgroups of LSc (generalized morphoea, 89%; linear scleroderma, 71% ; morphoea, 90%). Patients with systemic lupus erythematosus (SLE) exhibited a similar frequency of antinucleosome antibody positivity (13 of 15, 87%), but th eir IgG levels of this autoantibody were much higher than those found in patient s with LSc. By contrast, IgM antinucleosome antibody levels were normal in patie nts with SLE, while they were significantly increased in patients with LSc compa red with normal controls. Antinucleosome antibody was also detected at lower fre quency in patients with systemic sclerosis(fiveof20,25%)ordermatomyositis(fiveo f15,33%). Nucleosome-restricted antibodies, i.e. antibodies that react with th e whole nucleosome particle but not with its individual components (histones and dsDNA) were also present in 35%of patients with LSc. Conclusions:Although ant inucleosome antibody was not specific to LSc, its high prevalence in LSc indicat es that antinucleosome antibody is a major autoantibody in this disease.
