Pemphigus vulgaris localized to the nose and cheeks
Pemphigus vulgaris localized to the nose and cheeks
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DOI：
10.1067/mjd.2002.121359
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发表时间：
2002-12-01
影响因子：
13.8
通讯作者：
Hertl, M
中科院分区：
文献类型：
--
作者：
Baykal, C;Azizlerli, G;Hertl, M
寻常型天疱疮是一种自身免疫性水疱性疾病，以弥漫性大疱性大疱病和粘膜表面及皮肤的侵蚀为特征。寻常型天疱疮在无毛皮肤上有局限性损害的病例已有报道，其中大部分被归因于Koebner现象。局限于鼻子的损害大多在叶状天疱疮中被描述，但在少数患者中，鼻子也是寻常型天疱疮的初始部位。我们报告4例局限性寻常型天疱疮：1例局限于鼻和脸颊，3例仅见于鼻部。这些患者的反复发作也发生在鼻子上。随访2~9年，无一例出现黏膜受累或播散。免疫印迹法检测到3例患者有抗桥粒蛋白-3的自身抗体。另1例采用酶联免疫吸附试验检测桥粒芯糖蛋白-3抗体。这些局限性损害可能代表寻常型天疱疮的一个亚群或在这种慢性病期间的一段有限活动期。
Pemphigus vulgaris is an autoimmune blistering disease characterized by disseminated bullae and erosions of the mucosal surfaces and skin. Cases of pemphigus vulgaris with localized lesions on glabrous skin have been reported, and most of them have been attributed to the Koebner phenomenon. Lesions limited to the nose have mostly been described in pemphigus foliaceus, but the nose has also been the initial site of pemphigus vulgaris in a few patients. We report 4 cases of localized pemphigus vulgaris: one case with lesions limited to the nose and cheeks and 3 cases with isolated lesions on the nose. Recurrent episodes in these patients also occurred on the nose. None of them showed mucosal involvement or dissemination during a follow-up period of 2 to 9 years. Three patients had autoantibodies against desmoglein-3 as detected by immunoblot. In the other patient, antibodies against desmoglein-3 were detected by enzyme-linked immunosorbent assay. These localized lesions may represent a subgroup of pemphigus vulgaris or a period of limited activity during this chronic disease.