Moussaoui Eya, Oueslati Yassine, Oualha Lamia, Denguezli Mohamed, Sriha Badreddine, Douki Nabiha
Department of Dental Medicine, Sahloul Hospital (Sousse), Dental Faculty of Monastir, University of Monastir, Tunisia.
Laboratory of Oral Health and Maxillofacial Rehabilitation (LR12ES11), University of Monastir, Tunisia.
Case Rep Dent. 2021 Oct 25;2021:7792360. doi: 10.1155/2021/7792360. eCollection 2021.
The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors' knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.
寻常型天疱疮（PV）在自身免疫性大疱性疾病（AIBD）中的地位已得到充分确立。它是一种后天性慢性自身免疫性水疱大疱性疾病，其中IgG抗体靶向桥粒蛋白以产生上皮内黏膜皮肤水疱。对于临床医生来说，诊断通常具有挑战性。它需要结合三个主要特征：临床、组织病理学和免疫学特征。临床上，在50 - 90%有广泛水疱累及口腔黏膜的患者中，口腔病变是该病的首发表现。在皮肤上，病变的特征是松弛性水疱，迅速发展为糜烂和结痂形成。脐部病变作为PV的一种临床表现较为特殊，很少有报道，且尚未完全阐明。在一项仅纳入10例患者的有限研究中，对天疱疮患者的脐部受累情况进行了评估。这种定位可能是一个有价值的线索，有助于在临床层面为有口腔黏膜水疱的患者进行诊断。牙医必须熟悉PV的临床表现，以便早期诊断并开始早期治疗，这决定了疾病的预后。据作者所知，仅在1例增殖型天疱疮（PVe）病例中报道了这些病变与口腔病变同时作为PV的首发体征且无皮肤受累的情况。在本文中，我们描述了一例女性患者的观察情况，该患者被诊断为PV，最初同时出现口腔和脐部病变，在其他皮肤病变出现前共存了4个月。我们强调牙医熟悉PV临床表现的作用，以便早期诊断、开始早期治疗（这决定了疾病的预后）以及密切跟踪病变的演变，必要时改变治疗方案。我们还讨论了该疾病的临床、组织学和免疫学特征，这些特征有助于进行鉴别诊断以及采取适当的治疗管理。
