Baykal Can, Azizlerli Gülsevim, Thoma-Uszynski Sybille, Hertl Michael
Department of Dermatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
J Am Acad Dermatol. 2002 Dec;47(6):875-80. doi: 10.1067/mdj.2002.121359.
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.
寻常型天疱疮是一种自身免疫性水疱病，其特征为黏膜表面和皮肤出现散在的水疱和糜烂。已有报道称寻常型天疱疮患者在无毛皮肤上出现局限性病变，其中大多数归因于同形反应。局限于鼻部的病变大多在落叶型天疱疮中有所描述，但在少数患者中，鼻子也是寻常型天疱疮的初始发病部位。我们报告4例局限性寻常型天疱疮病例：1例病变局限于鼻子和脸颊，3例仅鼻子有孤立性病变。这些患者的复发也发生在鼻子部位。在2至9年的随访期内，他们均未出现黏膜受累或病变播散。通过免疫印迹法检测，3例患者存在抗桥粒芯糖蛋白-3自身抗体。在另一例患者中，通过酶联免疫吸附测定法检测到抗桥粒芯糖蛋白-3抗体。这些局限性病变可能代表寻常型天疱疮的一个亚组，或者是这种慢性病的一个活动受限阶段。
