Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus.
Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus.
复制标题
副肿瘤性天疱疮的临床表型和抗桥粒芯糖蛋白自身抗体谱。
DOI：
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发表时间：
2001
期刊：
影响因子：
--
通讯作者：
T. Nishikawa
中科院分区：
文献类型：
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作者：
Manabu Ohyama;M. Amagai;Takashi Hashimoto;H. Nousari;G. Anhalt;T. Nishikawa
背景
副肿瘤性天疱疮(PNP)具有与寻常型天疱疮(PV)相似的特征，包括循环中的抗桥粒芯糖蛋白(DSG)Ig G作为致病自身抗体。将PV分为黏膜显性型和黏膜皮肤型时，粘膜显性型仅有抗Dsg3抗体，而黏膜皮肤型既有抗Dsg3抗体又有抗DSG1抗体。
客观化
本研究的目的是确定粘膜显性PNP和皮肤粘膜PNP之间是否存在抗DSG自身抗体谱的差异。
方法
根据临床资料，21例PNP患者被分为黏膜优势型和黏膜皮肤型。以重组DSG1和Dsg3为载体，采用酶联免疫吸附试验检测Dsg3和DSG1抗体效价。
结果
黏膜显性型9例，黏膜皮肤型12例。9例粘膜显性型中8例抗Dsg3抗体阳性，3例同时抗DSG1抗体阳性。12例黏膜皮肤型患者抗Dsg3抗体均为阳性，仅6例抗DSG1抗体阳性。
结论
在PNP中，临床表型与抗DSG抗体谱之间没有明显的关联，而在PV中则是如此。这一发现提示，除抗DSG抗体外，其他病理机制，如苔藓样反应或界面性皮炎可能参与了PNP中水泡的形成。
BACKGROUND
Paraneoplastic pemphigus (PNP) has similar features to pemphigus vulgaris (PV), including circulating anti-desmoglein (Dsg) IgG as pathogenic autoantibodies. When PV is divided into mucosal dominant type and mucocutaneous type, mucosal dominant type has only anti-Dsg3 IgG, whereas the mucocutaneous type has both anti-Dsg3 and anti-Dsg1 IgG.
OBJECTIVE
The purpose of this study was to determine whether there is a difference in anti-Dsg autoantibody profile between mucosal dominant PNP and mucocutaneous PNP.
METHODS
Twenty-one patients with PNP were categorized as mucosal dominant and mucocutaneous types based on clinical information. Antibody titers against Dsg3 and Dsg1 were measured by enzyme-linked immunosorbent assay by means of recombinant Dsg1 and Dsg3.
RESULTS
There were 9 cases of mucosal dominant type and 12 cases of mucocutaneous type. Eight of 9 cases of mucosal dominant type were positive for anti-Dsg3 IgG, but 3 of them were also positive for anti-Dsg1 IgG. All 12 cases of mucocutaneous type were positive for anti-Dsg3 IgG, whereas only 6 of them were positive for anti-Dsg1 IgG.
CONCLUSION
There was no clear association between the clinical phenotype and anti-Dsg antibody profile in PNP as seen in PV. This finding suggests that besides anti-Dsg IgG other pathologic mechanisms such as lichenoid reaction or interface dermatitis may be involved in the blister formation in PNP.
影响因子：
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作者：
T. Horn;G. Anhalt
通讯作者：
T. Horn;G. Anhalt
姑息性放射治疗后副肿瘤性天疱疮表现为大疱性类天疱疮样皮疹。
影响因子：
13.8
作者：
Fried,R;Lynfield,Y;Vitale,P;Anhalt,G
通讯作者：
Anhalt,G
影响因子：
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作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper
通讯作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper