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口腔黏膜类天疱疮和寻常型天疱疮——一项回顾性双中心队列研究。

Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study.

作者信息

Sultan A S, Villa A, Saavedra A P, Treister N S, Woo S-B

机构信息

Division of Oral Medicine and Dentistry, Brigham and Women's Hospital, Boston, MA, USA.

Department of Oral Medicine, Infection and Immunity, Harvard School of Dental Medicine, Boston, MA, USA.

出版信息

Oral Dis. 2017 May;23(4):498-504. doi: 10.1111/odi.12639. Epub 2017 Feb 22.

Abstract

OBJECTIVES

Few studies have compared oral mucous membrane pemphigoid (MMP) and pemphigus vulgaris (PV). Descriptive analysis of oral features, extent of extra-oral involvement, and management outcomes were performed.

SUBJECTS AND METHODS

Patients with PV and MMP, the latter with exclusive oral involvement at first presentation, were included.

RESULTS

There were 26 MMP (46%) and 31 PV (54%) patients. Desquamative gingivitis was evident in 84% of MMP cases compared to 28% of PV cases (P < 0.05). Non-gingival lesions were noted in 6% of MMP cases compared to 55% of PV cases (P < 0.01). Management of MMP consisted of only topical corticosteroids in 88% of cases while 12% of cases required concomitant systemic therapy. All PV cases (100%) required systemic therapy. No patients with MMP developed scarring or ocular lesions, and one patient (4%) developed cutaneous lesions. Five PV cases (16%) had oral cavity involvement only with three (60%) developing pharyngeal involvement and two (40%) developing cutaneous lesions on follow-up.

CONCLUSION

Oral MMP presents primarily as desquamative gingivitis, infrequently involving extragingival sites, and is highly amenable to topical therapy, while PV is a systemic mucocutaneous disease with extensive non-gingival oral lesions that almost always requires systemic therapy.

摘要

目的

很少有研究比较口腔黏膜类天疱疮(MMP)和寻常型天疱疮(PV)。对口腔特征、口腔外累及范围及治疗结果进行了描述性分析。

研究对象与方法

纳入PV和MMP患者,后者初诊时仅累及口腔。

结果

共有26例MMP患者(46%)和31例PV患者(54%)。84%的MMP病例出现剥脱性龈炎,而PV病例为28%(P<0.05)。6%的MMP病例出现非牙龈病变,而PV病例为55%(P<0.01)。88%的MMP病例治疗仅采用局部皮质类固醇,12%的病例需要联合全身治疗。所有PV病例(100%)均需要全身治疗。MMP患者均未出现瘢痕形成或眼部病变,1例患者(4%)出现皮肤病变。5例PV病例(16%)初诊时仅累及口腔,随访时3例(60%)出现咽部累及,2例(40%)出现皮肤病变。

结论

口腔MMP主要表现为剥脱性龈炎,很少累及龈外部位,且对局部治疗高度敏感,而PV是一种系统性黏膜皮肤疾病,有广泛的非牙龈口腔病变,几乎总是需要全身治疗。

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