口面部肉芽肿病的治疗：一例报告
Treatment of orofacial granulomatosis: a case report.
作者信息
Badshah Maaz B, Walayat Saqib, Ahmed Umair, Dhillon Sonu, Yong Sherri, Kane Sunanda, Thievanayagam Shoba
机构信息
Department of Gastroenterology and Hepatology, University of Illinois Peoria Campus, OSF Saint Francis Medical Center, Peoria, IL, 61637, USA.
Department of Internal Medicine, University of Illinois Peoria Campus, OSF Saint Francis Medical Center, Peoria, IL, 61637, USA.
出版信息
J Med Case Rep. 2017 Oct 25;11(1):300. doi: 10.1186/s13256-017-1455-4.
BACKGROUND
Orofacial granulomatosis is a relatively recent term coined by Wiesenfield et al. in 1985 to define granulomatous lesions of oral mucosa without intestinal involvement. When it presents in a triad encompassing facial nerve palsy, lip swelling, and fissured or furrowed tongue it is called Melkersson-Rosenthal syndrome while monosymptomatic or oligosymptomatic forms are referred to as granulomatous cheilitis. It is an uncommon clinicopathologic entity which is distinct from classic Crohn's disease. The NOD2 variant which is commonly associated with Crohn's has not been shown to have any association with orofacial granulomatosis.
CASE PRESENTATION
We present a case of a 31-year-old white man who had painful swelling of the lip with oral ulcers and difficulty eating for 2 to 3 years. He was diagnosed as having granulomatous cheilitis based on characteristic biopsy findings. There was serologic evidence of Crohn's disease with anti-Saccharomyces cerevisiae antibodies. However, he was not found to have any gastrointestinal involvement based on computed tomography enterography, and upper and lower endoscopies. He failed to respond to nonsteroidal anti-inflammatory drugs, steroids, and dapsone therapy but responded well to high doses of infliximab.
CONCLUSIONS
Our case questions whether granulomatous cheilitis really exists or is it simply a variant of Crohn's disease with only oral presentation. Our patient did not have symptoms of Crohn's disease; moreover, endoscopic studies and computed tomography enterography were unremarkable for evidence of intestinal involvement. Our case is also the first reported case where high-dose infliximab alone has been used with sustained response for approximately 8 months. In conclusion, more research is needed to assess the underlying pathology as well as ideal treatment options for patients with orofacial granulomatosis. We propose that high-dose infliximab should be considered in patients who do not respond to traditional therapies.
背景
口面部肉芽肿病是1985年由维森菲尔德等人提出的一个相对较新的术语，用于定义无肠道受累的口腔黏膜肉芽肿性病变。当它以包括面神经麻痹、唇部肿胀以及舌部皲裂或沟纹的三联征形式出现时，被称为梅尔克森 - 罗森塔尔综合征，而单症状或少症状形式则被称为肉芽肿性唇炎。它是一种罕见的临床病理实体，与经典克罗恩病不同。常见于克罗恩病的NOD2变异体尚未被证明与口面部肉芽肿病有任何关联。
病例介绍
我们报告一例31岁白人男性病例，其唇部疼痛肿胀伴口腔溃疡，进食困难2至3年。基于特征性活检结果，他被诊断为肉芽肿性唇炎。有抗酿酒酵母抗体的血清学证据支持克罗恩病。然而，根据计算机断层扫描小肠造影、上下消化道内镜检查，未发现他有任何胃肠道受累情况。他对非甾体类抗炎药、类固醇和氨苯砜治疗无反应，但对高剂量英夫利昔单抗反应良好。
结论
我们的病例引发了关于肉芽肿性唇炎是否真的存在，还是仅仅是克罗恩病仅累及口腔的一种变体的疑问。我们的患者没有克罗恩病的症状；此外，内镜检查和计算机断层扫描小肠造影未发现肠道受累的证据。我们的病例也是首例单独使用高剂量英夫利昔单抗且持续反应约8个月的报道病例。总之，需要更多研究来评估口面部肉芽肿病的潜在病理以及理想的治疗方案。我们建议，对于对传统疗法无反应的患者应考虑使用高剂量英夫利昔单抗。
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