Archibald Curtis W, Punja Karim G, Oryschak Allan F
Department of Surgery, Division of Ophthalmology, University of Calgary, Calgary, Alberta, Canada.
Saudi J Ophthalmol. 2012 Apr;26(2):177-9. doi: 10.1016/j.sjopt.2012.02.009. Epub 2012 Mar 3.
Orofacial granulomatosis (OFG) is an uncommon but increasingly recognized disease of unknown etiology. The typical presentation is chronic swelling of the perioral soft tissue, but eyelid edema can be the sole manifestation. Terminology of this disease can be confusing as it may also be referred to as granulomatous cheilitis and a monosymptomatic presentation of Melkersson-Rosenthal syndrome (MRS). Crohn's disease and sarcoidosis should also be considered in the differential as the histopathology can be similar. Corticosteroids are the mainstay of treatment but can lack efficacy. Atypical presentations and the possibility of systemic disease involvement can further challenge the management. We describe an unusual case in which OFG manifests solely as chronic eyelid swelling. This 69-year old Asian female patient had a delayed diagnosis that responded well to intralesional corticosteroid injection with surgical skin reduction. In addition to describing this unusual presentation of OFG, we review the relevant literature and evaluate the current terminology used to describe this entity.
口面部肉芽肿病(OFG)是一种病因不明的罕见疾病,但越来越受到认可。典型表现为口周软组织慢性肿胀,但眼睑水肿也可能是唯一表现。该疾病的术语可能会令人困惑,因为它也可能被称为肉芽肿性唇炎以及梅尔克森 - 罗森塔尔综合征(MRS)的单症状表现。鉴别诊断时还应考虑克罗恩病和结节病,因为它们的组织病理学可能相似。皮质类固醇是主要治疗方法,但可能缺乏疗效。非典型表现以及全身性疾病受累的可能性会给治疗带来进一步挑战。我们描述了一例不寻常的病例,其中OFG仅表现为慢性眼睑肿胀。这位69岁的亚洲女性患者诊断延迟,经病灶内注射皮质类固醇及手术皮肤缩减后反应良好。除了描述OFG的这种不寻常表现外,我们还回顾了相关文献并评估了用于描述该实体的当前术语。