Oral Medicine, University College London Eastman Dental Institute and University College London Hospital Eastman Dental Hospital, London, UK.
J Am Acad Dermatol. 2010 Apr;62(4):611-20. doi: 10.1016/j.jaad.2009.03.051. Epub 2010 Feb 4.
Orofacial granulomatosis (OFG) is a chronic inflammatory disorder characterized by persistent or recurrent soft tissue enlargement, oral ulceration, and a variety of other orofacial features. There remain few detailed reports of the clinical features and long-term response to therapy of substantial groups of patients with OFG.
The aim of this study was to determine retrospectively the clinical, hematologic, and histopathological features of a large case series of patients with OFG. In addition the long-term response to therapy was examined.
Clinically relevant data of 49 patients with OFG who attended a single oral medicine unit in the United Kingdom were retrospectively examined. The analyzed parameters included diagnostic features, clinical manifestations, and outcomes and adverse side effects of therapy.
Labial swelling was the most common presenting clinical feature at diagnosis (75.5%), followed by intraoral mucosal features other than ulceration such as cobblestoning and gingival enlargement (73.5%). Mucosal ulceration was observed in 36.7% of patients whereas extraoral facial manifestations such as cutaneous erythema and swelling were present in 40.8% of patients. Of the 45 patients who required treatment, 24 (53.3%) were treated with topical corticosteroids/immunosuppressants only, whereas 21 (46.7%) received a combined therapy (topical plus systemic corticosteroids/immunosuppressants and/or intralesional corticosteroids). The long-term outcome analysis showed complete/partial resolution of tissue swelling and oral ulceration in 78.8% and 70% of patients, respectively.
The main limitation of the current study was its retrospective design and methodology including differences in reporting clinical features and outcome.
OFG can show multiple facial and mucosal clinical features. Long-term treatment with topical and/or combined therapy is needed in the majority of patients. Response to therapy is highly variable even though in the long-term complete/partial disease resolution can be obtained in the majority of patients. Mucosal ulceration tends to be more recalcitrant than orofacial swelling. Adverse side effects of therapy are rare.
口腔面肉芽肿病（OFG）是一种慢性炎症性疾病，其特征为持续或复发性软组织肿大、口腔溃疡和多种其他口腔面特征。目前关于大量 OFG 患者的临床特征和长期治疗反应的详细报告很少。
本研究旨在回顾性确定一大组 OFG 患者的临床、血液学和组织病理学特征。此外，还检查了治疗的长期反应。
回顾性检查了在英国单一口腔医学科就诊的 49 例 OFG 患者的临床相关数据。分析的参数包括诊断特征、临床表现以及治疗的结局和不良反应。
唇肿胀是诊断时最常见的临床表现（75.5%），其次是口腔内非溃疡性黏膜特征，如鹅卵石样外观和牙龈肿大（73.5%）。黏膜溃疡见于 36.7%的患者，而面部皮肤红斑和肿胀等眶周面部表现见于 40.8%的患者。在需要治疗的 45 例患者中，24 例（53.3%）仅接受局部皮质类固醇/免疫抑制剂治疗，21 例（46.7%）接受联合治疗（局部加全身皮质类固醇/免疫抑制剂和/或皮损内皮质类固醇）。长期结局分析显示，组织肿胀和口腔溃疡的完全/部分缓解率分别为 78.8%和 70%。
本研究的主要局限性是其回顾性设计和方法，包括临床特征和结局的报告存在差异。
OFG 可表现出多种面部和黏膜临床特征。大多数患者需要长期接受局部和/或联合治疗。即使在长期治疗中，大多数患者也能获得完全/部分疾病缓解，但治疗反应具有高度变异性。黏膜溃疡比口腔面肿胀更顽固。治疗的不良反应罕见。
