Oral Medicine, Dental Institute, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Centre for Host-Microbiome Interactions, King's College London, Faculty of Dentistry, Oral and Craniofacial Sciences, London, UK.
Clin Exp Dermatol. 2019 Oct;44(7):732-739. doi: 10.1111/ced.13996. Epub 2019 May 18.
The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history-taking, clinical examination, an understanding of pathogenesis and appropriate investigations are essential. With the exception of the rare genodermatoses that may lead to blistering and oral ulceration, the majority of patients have an acquired disorder. These include the rare autoimmune blistering diseases mucous membrane pemphigoid (MMP), pemphigus vulgaris (PV), linear IgA disease, epidermolysis bullosa acquisita and paraneoplastic pemphigus. Important clinical differential diagnoses include erythema multiforme, which may be mistaken for PV in appearance, while oral lichen planus may be indistinguishable from MMP. Angina bullosa haemorrhagica may also present with tense haemorrhagic bullae, and in the absence of diagnostic tests, requires an astute clinical diagnosis based upon the history. Newer laboratory techniques have facilitated identification of target antigens and epitopes in the autoimmune blistering diseases, particularly in MMP. Current interest is in whether these relate to clinical presentation and outcomes. There have also been recent investigations into the use of saliva as an alternative medium to serum for the diagnosis of oral vesiculobullous lesions. Assessment of disease severity and measurement of quality of life at presentation and subsequent follow-up is paramount to interpreting therapeutic response. Furthermore, combining these scores with serological and/or salivary biomarkers is valuable in the assessment of clinical response. In this paper, we discuss MMP and its important differential diagnoses.
自身免疫性水疱性疾病在口腔中以不同的频率出现。在出现时识别其关键临床特征非常重要,因为口腔溃疡有许多原因。仔细的病史采集、临床检查、对发病机制的理解和适当的检查是必不可少的。除了极少数可能导致水疱和口腔溃疡的遗传性皮肤病外,大多数患者都患有获得性疾病。这些疾病包括罕见的自身免疫性水疱性疾病黏膜类天疱疮 (MMP)、寻常性天疱疮 (PV)、线性 IgA 病、获得性大疱性表皮松解症和副肿瘤性天疱疮。重要的临床鉴别诊断包括多形红斑,其外观可能被误诊为 PV,而口腔扁平苔藓可能与 MMP 无法区分。疱疹样口炎也可能出现紧张性出血性水疱,如果没有诊断性检查,则需要根据病史进行敏锐的临床诊断。新的实验室技术促进了自身免疫性水疱性疾病中靶抗原和表位的识别,尤其是在 MMP 中。目前的兴趣在于这些是否与临床表现和结果有关。最近还对唾液作为替代血清诊断口腔水疱性病变的介质进行了研究。在出现时评估疾病严重程度和生活质量,并在随后的随访中进行评估,对于解释治疗反应至关重要。此外,将这些评分与血清学和/或唾液生物标志物相结合,对于评估临床反应非常有价值。在本文中,我们讨论了 MMP 及其重要的鉴别诊断。