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韦尔斯综合征表现为不典型眶周蜂窝织炎。

Wells Syndrome Presenting as Atypical Periorbital Cellulitis.

机构信息

Johns Hopkins University School of Medicine, Baltimore, Maryland.

Department of Ophthalmology, Ophthalmic Plastic Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts.

出版信息

Ophthalmic Plast Reconstr Surg. 2022;38(6):e167-e170. doi: 10.1097/IOP.0000000000002220. Epub 2022 Jun 14.

DOI:10.1097/IOP.0000000000002220
PMID:35699219
Abstract

A 62-year-old man presented with diffuse, painless, left-sided preseptal edema, erythema, and woody induration extending to the left temple. The induration generated an orbital compartment syndrome with markedly elevated intraocular pressure necessitating lateral canthotomy and cantholysis. Although atypical for an infectious etiology, empiric broad-spectrum intravenous antibiotics were initiated with no improvement. A tissue biopsy demonstrated extensive perivascular and interstitial eosinophils with focal flame figures, and the patient was diagnosed with a severe hypersensitivity reaction or eosinophilic cellulitis (Wells syndrome). The disease process remitted rapidly upon initiation of oral prednisone. Wells syndrome is a rare inflammatory eosinophilic dermatosis, most often presenting in the limbs and trunk, with few reports of facial and periorbital involvement. This case highlights the importance of considering Wells syndrome in the differential diagnosis of atypical periorbital cellulitis that is nonresponsive to antibiotics and reviews the clinicopathologic nature of this disease.

摘要

一位 62 岁男性因弥漫性、无痛性、左侧眶隔前水肿、红斑和累及左侧太阳穴的木样硬结而就诊。硬结导致眶隔间隙综合征,眼内压显著升高,需要进行外侧眦切开和眦松解术。尽管这种表现不太典型,但仍经验性地给予了广谱静脉内抗生素治疗,但无改善。组织活检显示广泛的血管周围和间质嗜酸性粒细胞,伴有局灶性火焰状图形,因此诊断为严重过敏反应或嗜酸性粒细胞性蜂窝织炎(韦尔斯综合征)。口服泼尼松后,疾病过程迅速缓解。韦尔斯综合征是一种罕见的炎症性嗜酸性粒细胞皮肤病,最常发生在四肢和躯干,很少有面部和眶周受累的报道。本病例强调了在对非抗生素治疗反应不佳的非典型眶周蜂窝织炎进行鉴别诊断时,应考虑韦尔斯综合征的重要性,并回顾了该病的临床病理特征。

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