背景:寻常型天疱疮(PV)是一种可能危及生命的皮肤粘膜自身免疫性疾病,会影响桥粒蛋白-1和桥粒蛋白-3,导致上皮内囊泡病变。在口腔粘膜中,PV病变可以模仿其他疾病,如粘膜类天疱疮,其他形式的天疱疮,复发性口疮性口炎,多形性红斑,史蒂文斯-约翰逊综合征,和病毒诱导的溃疡,如单纯疱疹病毒(HSV),诊断具有挑战性。PV与克罗恩病的共同发生很少见,主要见于年轻患者。PV和克罗恩病的主要治疗方法通常包括全身性皮质类固醇与免疫抑制剂和免疫生物学药物的组合。文献表明,使用这些药物,特别是TNF-α抑制剂,用于管理自身免疫性疾病,如克罗恩病可以潜在地诱发其他称为自身免疫样综合征的自身免疫性疾病,其中包括狼疮样综合征和炎性神经病的发作。文献中很少有病例报道接受英夫利昔单抗治疗的CD患者中PV的发展。
方法:一名患有严重克罗恩病的年轻女性,用TNF-α抑制剂英夫利昔单抗治疗,出现脆性假膜性口腔溃疡。组织病理学和免疫荧光分析证实这些为PV。治疗包括丙酸氯倍他索和低水平光生物调节,这导致了部分改进。患者后来出现严重的肠出血,需要静脉注射氢化可的松治疗,改善了她的全身状况和口腔病变。几周后,发现了由疱疹病毒和念珠菌病引起的新的溃疡,导致口服阿昔洛韦治疗,21天的口服制霉菌素冲洗方案,光动力疗法,最终治愈口腔感染。为了控制她的病情,胃肠病学家在她的治疗方案中包括甲氨蝶呤(25毫克),以降低英夫利昔单抗的免疫原性并尽量减少皮质类固醇的使用,因为病人正在缓解克罗恩病,口腔PV病变得到控制。
结论:患有克罗恩病的年轻患者应转诊至口腔医学专家进行合并症调查,因为免疫抑制治疗期间可能出现口腔PV和机会性感染。在治疗炎症性肠病的患者中使用TNF-α抑制剂,比如克罗恩,应该仔细评估潜在的副作用,包括口服PV。
BACKGROUND: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn\'s disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn\'s disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn\'s can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy.
METHODS: A young female with severe Crohn\'s disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn\'s disease, and the oral PV lesions were under control.
CONCLUSIONS: Young patients with Crohn\'s disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn\'s, should be carefully evaluated for potential side effects, including oral PV.