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中毒性表皮坏死松解症患者静脉注射免疫球蛋白治疗后的皮肤免疫球蛋白沉积

Skin immunoglobulin deposition following intravenous immunoglobulin therapy in toxic epidermal necrolysis.

作者信息

Paquet P, Kaveri S, Jacob E, Pirson J, Quatresooz P, Piérard G E

机构信息

Department of Dermatopathology, University Hospital of Liège, Liège, Belgium.

出版信息

Exp Dermatol. 2006 May;15(5):381-6. doi: 10.1111/j.0906-6705.2006.00426.x.

DOI:10.1111/j.0906-6705.2006.00426.x
PMID:16630079
Abstract

Human intravenous immunoglobulins (IVIg) which contain anti-CD95 antibodies have been proposed to treat toxic epidermal necrolysis (TEN). Presently, there is no evidence that IVIg reach the keratinocytes in TEN patients. The aim of this study was to assess the Ig distribution in the serum, blister fluid and skin of six consecutive TEN patients treated with IVIg (1 g/kg/day) for 3 days. They were compared with five TEN patients who only received supportive therapy. In all patients, IgA, IgM and IgG concentrations were measured in the serum and blister fluid using an immuno-nephelometric method. Immunohistochemistry was performed on skin biopsies taken from both TEN clinically involved and uninvolved skin to search for IgG deposits. On admission, the IgG concentrations were significantly higher in both TEN serum and TEN blister fluid compared with their respective IgA and IgM contents. The IgG, IgA and IgM concentrations in blister fluid were significantly lower than their respective serum concentrations. The serum and blister fluid IgG concentrations, but not that of IgA and IgM, were markedly increased at the completion of the IVIg treatment. By contrast, they remained unchanged in the TEN patients that were untreated with IVIg. In the IVIg-treated patients, the IgG intraepidermal deposits raised markedly in both TEN-involved and uninvolved skin. This was not the case in patients who did not receive IVIg. These results suggest that IVIg perfusions brought a prominent increase in IgG concentration in the serum, blister fluid and epidermis of both TEN-involved and clinically uninvolved skin. The presence of potentially protective IgG in TEN epidermis following IVIg treatment could help limiting the disease progression.

摘要

含有抗CD95抗体的人静脉注射免疫球蛋白(IVIg)已被提议用于治疗中毒性表皮坏死松解症(TEN)。目前,尚无证据表明IVIg能到达TEN患者的角质形成细胞。本研究的目的是评估连续6例接受IVIg(1 g/kg/天)治疗3天的TEN患者血清、水疱液和皮肤中的免疫球蛋白分布情况。将他们与5例仅接受支持治疗的TEN患者进行比较。对所有患者,采用免疫比浊法测定血清和水疱液中的IgA、IgM和IgG浓度。对取自TEN临床受累皮肤和未受累皮肤的皮肤活检组织进行免疫组织化学检测,以寻找IgG沉积。入院时,TEN患者血清和水疱液中的IgG浓度显著高于各自的IgA和IgM含量。水疱液中的IgG、IgA和IgM浓度显著低于各自的血清浓度。IVIg治疗结束时,血清和水疱液中的IgG浓度显著升高,但IgA和IgM浓度未升高。相比之下,未接受IVIg治疗的TEN患者其浓度保持不变。在接受IVIg治疗的患者中,TEN受累皮肤和未受累皮肤的表皮内IgG沉积均显著增加。未接受IVIg治疗的患者则不然。这些结果表明,IVIg灌注使TEN受累皮肤和临床未受累皮肤的血清、水疱液和表皮中的IgG浓度显著升高。IVIg治疗后TEN表皮中存在潜在的保护性IgG可能有助于限制疾病进展。

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