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[由浅表剥脱性龈炎引发的瘢痕性类天疱疮。1例临床及免疫电子显微镜研究]

[Cicatricial pemphigoid disclosed by superficial desquamative gingivitis. Clinical and immuno-electron microscopic study of a case].

作者信息

Vaillant L, Arbeille B, Goga D, de Muret A, Prime A, Lorette G

机构信息

Service de Dermatologie, CHU Trousseau, Tours.

出版信息

Ann Dermatol Venereol. 1990;117(9):613-20.

PMID:2260801
Abstract

Six months before consulting, a 71-year old man developed buccal and genital erosions which gradually became worse. Physical examination showed signs of superficial desquamative gingivitis, wide erosions on the bony palate, erythemato-erosive balanoposthitis and 7 bullae or skin erosions on the upper part of the back. At histopathological examination of a cutaneous bulla there was dermoepidermal cleavage and an inflammatory infiltrate without eosinophils. At direct immunofluorescence, linear deposits of IgG and C3 were present along the basement membrane. A search for anti-skin autoantibodies was negative at indirect immunofluorescence but positive at immunoblotting (240 Kd band). The cicatricial pemphigoid was treated with dapsone alone in doses of 100 mg/day. Treatment was continued for 6 months, resulting in complete cure of the mucosal and cutaneous lesions. An immunoelectromicroscopic study, performed according to the technique described by Prost et al., on a fragment of skin from around the bullae, showed deposits of granular IgG in the lamina lucida and the lamina densa and deposits of C3 in the lamina densa. This case of cicatricial pemphigoid exhibited 3 features which are not usually found in bullous pemphigoid. Clinically, the buccal lesions were located on the gums and on the hard palate, i.e. where the mucosa adheres to the underlying bone through the periosteum. This location is habitual in cicatricial pemphigoid and differs from that of the bullous pemphigoid lesions which affect the free mucosa lining the cheeks and the soft palate. Treatment with dapsone was dramatically successful in our patient whose lesions disappeared in 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

在就诊前六个月,一名71岁男性出现颊部和生殖器糜烂,且逐渐加重。体格检查显示有浅表性剥脱性牙龈炎体征、硬腭广泛糜烂、红斑糜烂性阴茎头炎以及背部上方7处大疱或皮肤糜烂。对一处皮肤大疱进行组织病理学检查时,发现真皮表皮分离且无嗜酸性粒细胞的炎性浸润。直接免疫荧光检查显示,沿基底膜有IgG和C3的线性沉积。间接免疫荧光法检测抗皮肤自身抗体为阴性,但免疫印迹法检测呈阳性(240 Kd条带)。瘢痕性类天疱疮仅用氨苯砜治疗,剂量为每日100毫克。治疗持续6个月,黏膜和皮肤病变完全治愈。按照Prost等人描述的技术,对大疱周围的皮肤碎片进行免疫电镜研究,结果显示在透明层和致密层有颗粒状IgG沉积,在致密层有C3沉积。该例瘢痕性类天疱疮表现出3个通常在大疱性类天疱疮中未发现的特征。临床上,颊部病变位于牙龈和硬腭,即黏膜通过骨膜附着于下方骨骼的部位。此部位在瘢痕性类天疱疮中常见,与大疱性类天疱疮病变部位不同,后者影响脸颊和软腭的游离黏膜。氨苯砜治疗对我们的患者非常成功,其病变在6个月内消失。(摘要截断于250字)

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