Long-lasting Localized Pemphigus Vulgaris without Detectable Serum Autoantibodies Against Desmoglein 3 and Desmoglein 1.
Long-lasting Localized Pemphigus Vulgaris without Detectable Serum Autoantibodies Against Desmoglein 3 and Desmoglein 1.
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DOI：
10.4103/0019-5154.185712
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发表时间：
2016-07
影响因子：
1.7
通讯作者：
Kanekura T
中科院分区：
文献类型：
--
作者：
Yoshifuku A;Fujii K;Kawahira H;Katsue H;Baba A;Higashi Y;Aoyama Y;Kanekura T
寻常天疱疮（PV）是由抗桥粒芯糖蛋白（DsG）3抗体引起的自身免疫性水疱性疾病。虽然皮肤病变往往分布在整个身体，但在某些患者中，它们仅限于有限的区域。我们报告两名患者谁提出了持久的本地PV检测不到抗DsG抗体后，遭受抗体阳性的系统性PV。两名患者的泼尼松龙（PSL）初始治疗均获得成功，但在PSL剂量减少后，脸颊或下唇出现局部复发。局部病灶活检显示基底上棘层松解，血清中未发现DsG抗体，局部免疫抑制治疗有效。根据我们的研究结果，我们认为，局部PV没有检测到的抗体可以发展后，系统性PV。
Pemphigus vulgaris (PV) is an autoimmune blistering disease elicited by anti-desmoglein (DsG) 3 antibody. Although skin lesions tend to be distributed over the entire body, in some patients, they are confined to a restricted area. We report two patients who presented with long-lasting localized PV without detectable anti-DsG antibodies after suffering antibody-positive systemic PV. Initial treatment with prednisolone (PSL) was successful in both patients, but a local relapse occurred on the cheek or lower lip after a reduction in the PSL dose. Biopsy of the localized lesions showed suprabasal acantholysis; no serum DsG antibodies were found. Local immunosuppression therapy was effective in both patients. Based on our findings, we suggest that localized PV without detectable antibodies can develop after systemic PV.