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黏膜类天疱疮的最新进展:一种异质性免疫介导的上皮下疱性疾病。

Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity.

作者信息

Scully C, Carrozzo M, Gandolfo S, Puiatti P, Monteil R

机构信息

Eastman Dental Institute for Oral Health Care Science and International Centre for Excellence in Dentistry, United Kingdom.

出版信息

Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999 Jul;88(1):56-68. doi: 10.1016/s1079-2104(99)70194-0.

DOI:10.1016/s1079-2104(99)70194-0
PMID:10442946
Abstract

Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.

摘要

大多数皮肤疾病的口腔受累情况与黏膜类天疱疮或扁平苔藓有关。黏膜类天疱疮是1997年6月在意大利都灵举行的一次欧洲研讨会的主题。本综述基于该研讨会。黏膜类天疱疮是一种主要发生于中老年后期的上皮下疱性疾病;女性略为多见。虽然黏膜类天疱疮以前被认为是一种单一的疾病,但现在很明显,一些上皮下疱性疾病可能会产生相似的临床表现,而且黏膜类天疱疮存在一系列变体,其抗体针对各种半桥粒成分或上皮基底膜成分。因此,使用了免疫介导的上皮下疱性疾病这一术语。仅根据临床情况对免疫介导的上皮下疱性疾病进行诊断和管理是不可能的;现在总是需要全面的病史、全身和口腔检查以及免疫染色活检,有时还需辅以其他检查。大多数口腔受累的黏膜类天疱疮患者表现为剥脱性龈炎,这是一种相当常见的症状,多见于中年或老年女性。口腔水疱和糜烂也可能发生,并且可能有尼氏征阳性。一些患者有其他复层鳞状上皮的病变,表现为结膜、鼻、食管、喉、外阴、阴茎或肛门受累。除了改善口腔卫生外,通常需要免疫调节——特别是免疫抑制——治疗来控制黏膜类天疱疮的口腔病变。没有单一的治疗方案能可靠地控制所有这些疾病。

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