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儿童黏膜类天疱疮累及口腔和生殖器黏膜 1 例罕见病例报告。

A rare case of childhood mucous membrane pemphigoid involving the oral and genital mucosa.

机构信息

Ponce Health Sciences University, Ponce, PR, USA.

Department of Dermatology, Mayo Clinic, Jacksonville, FL, USA.

出版信息

Pediatr Dermatol. 2021 Jul;38(4):904-907. doi: 10.1111/pde.14627. Epub 2021 May 11.

DOI:10.1111/pde.14627
PMID:33977558
Abstract

Mucous membrane pemphigoid (MMP) is a rare chronic immunobullous disease that involves the mucous membranes and may result in significant scarring and complications if diagnosis is delayed. MMP typically occurs in elderly patients, with very few cases reported in children. Here, we present a 12-year-old female patient with childhood-onset oral and genital MMP, clinically suspected to be lichen sclerosus, but eventually diagnosed as MMP after multiple supportive biopsies and confirmatory direct immunofluorescence. Although treatment was challenging, the combined use of systemic corticosteroids, dapsone, and mycophenolate mofetil was ultimately successful in achieving disease control.

摘要

黏膜类天疱疮(Mucous membrane pemphigoid,MMP)是一种罕见的慢性免疫性大疱病,累及黏膜,如果诊断延迟,可能导致严重的瘢痕和并发症。MMP 通常发生在老年患者中,儿童病例非常少见。在这里,我们报告了一例 12 岁女性儿童期口腔和生殖器 MMP 患者,临床疑似硬皮病样扁平苔藓,但最终通过多次支持性活检和确认性直接免疫荧光检查诊断为 MMP。尽管治疗具有挑战性,但联合使用全身皮质类固醇、氨苯砜和霉酚酸酯最终成功地控制了疾病。

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1
A rare case of childhood mucous membrane pemphigoid involving the oral and genital mucosa.儿童黏膜类天疱疮累及口腔和生殖器黏膜 1 例罕见病例报告。
Pediatr Dermatol. 2021 Jul;38(4):904-907. doi: 10.1111/pde.14627. Epub 2021 May 11.
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[Pemphigoid of the mucous membranes. The clinical, histopathological and immunological aspects and current therapeutic concepts].[黏膜类天疱疮。临床、组织病理学和免疫学方面以及当前的治疗理念]
Minerva Stomatol. 1996 Oct;45(10):455-63.
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[Mucous membrane pemphigoid: clinical manifestations and treatment with corticosteroids, dapsone and cyclophosphamide in 5 patients].[黏膜类天疱疮:5例患者的临床表现及使用皮质类固醇、氨苯砜和环磷酰胺的治疗]
Actas Dermosifiliogr. 2005 Jul-Aug;96(6):357-64. doi: 10.1016/s0001-7310(05)73091-x.
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Treatment of mucous membrane pemphigoid with the combination of mycophenolate mofetil, dapsone, and prednisolone: a case series.采用霉酚酸酯、氨苯砜和泼尼松联合治疗黏膜类天疱疮:病例系列研究。
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Management of mucous membrane pemphigoid in a joint oral medicine-dermatology clinic.口腔医学-皮肤科联合门诊中黏膜类天疱疮的治疗。
Clin Exp Dermatol. 2020 Aug;45(6):685-690. doi: 10.1111/ced.14188. Epub 2020 Mar 24.

引用本文的文献

1
Case report: Minimal manifestations of mucous membrane pemphigoid in a young adult.病例报告:一名年轻成年人黏膜类天疱疮的轻微表现
Front Med (Lausanne). 2022 Nov 17;9:1052145. doi: 10.3389/fmed.2022.1052145. eCollection 2022.
2
European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.欧洲皮肤病学会和性病学会发起的黏膜类天疱疮诊断和治疗欧洲指南(S3)- 第一部分。
J Eur Acad Dermatol Venereol. 2021 Sep;35(9):1750-1764. doi: 10.1111/jdv.17397. Epub 2021 Jul 10.