Clinical and immunological profiles in 17 Japanese patients with drug-induced pemphigus studied at Kurume University.
Clinical and immunological profiles in 17 Japanese patients with drug-induced pemphigus studied at Kurume University.
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久留米大学研究了 17 名日本药物性天疱疮患者的临床和免疫学特征。
DOI：
10.1111/bjd.12925
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发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Hashim
中科院分区：
文献类型：
--
作者：
Yoshimura K;Ishii N;Hamada T;Abe T;Ono F;Hashikawa K;Fukuda S;Ohyama B;Koga H;Sogame R;Teye K;Ochiai T;Nakajima H;Nakajima K;Iijima S;Kanzaki M;Kojima K;Nagatani T;Fujimoto W;Karashima T;Nakama T;Ohata C;Furumura M;Tsuruta D;Hashim
药物诱导的天疱疮(DIP)表现为天疱疮的临床、组织病理学和免疫学特征。目的了解DIP患者的临床和免疫学特征。方法我们研究了1997-2012年间在库鲁姆大学医院接受治疗或从其他医院就诊的17例日本DIP患者。结果17例DIP患者中8例表现为叶状天疱疮，3例表现为疱疹样天疱疹，6例表现为不典型的大疱性皮损。负责的药物有16例(布比拉明9例，d-青霉胺4例，西塔普利、硫普宁和卡托普利各1例)，以及1例非硫醇药物柳氮磺吡啶。通过ELISA和/或IB分析，9名患者仅与桥粒芯糖蛋白1(DSG1)反应，4名患者与DSG1和DSG3反应，4名患者没有特异性反应。通过对正常人表皮提取物的IB检测，除了与DSG1呈阳性反应外，4名未检测到恶性肿瘤的患者还对210 kDa的包膜蛋白和190 kDa的周斑蛋白表现出副肿瘤性天疱疮样反应。4例抗Dsg3抗体阳性，无粘膜损害。11例患者停药后痊愈，6例病程较长或难治性，有可能发展为真正的天疱疮。结论本研究表明，本研究的DIP患者大多表现为叶天疱疮表型，并伴有抗DSG1自身抗体，由含硫药物引起。
BackgroundDrug‐induced pemphigus (DIP) shows clinical, histopathological and immunological features of pemphigus. However, little is known about immunological profiles in DIP.ObjectivesTo characterize clinical and immunological profiles in patients with DIP.MethodsWe studied 17 Japanese patients with DIP who were treated at Kurume University Hospital or who consulted from other hospitals between 1997 and 2012. Complicated diseases, clinical and histopathological manifestations, responsible drugs and findings in immunofluorescence, enzyme‐linked immunosorbent assays (ELISAs), immunoblotting (IB) and prognosis were analysed.ResultsEight of the 17 patients with DIP showed pemphigus foliaceus‐like appearance, three showed pemphigus herpetiformis‐like appearance, and six showed atypical bullous lesions. Responsible drugs were thiol‐containing drugs in 16 patients (bucillamine in nine cases,d‐penicillamine in four cases, and cetapril, thiopronine and captopril in one patient each), and a nonthiol drug, sulfasalazine, in one patient. By ELISAs and/or IB analyses, nine patients reacted only with desmoglein 1 (Dsg1), four reacted with Dsg1 and Dsg3, and four showed no specific reactivity. By IB of normal human epidermal extracts, in addition to positive reactivity with Dsg1, four patients with no detectable malignancy showed paraneoplastic pemphigus‐like reactivity with the 210‐kDa envoplakin and the 190‐kDa periplakin. Four cases showed anti‐Dsg3 antibodies without mucosal lesions. While 11 cases recovered after discontinuation of the causative drugs, six patients had a very protracted or intractable disease course, and might develop true pemphigus.ConclusionsThe present study indicated that the majority of the patients with DIP studied showed a pemphigus foliaceus‐type phenotype with anti‐Dsg1 autoantibodies, caused by thiol‐containing drugs.