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罕见的 Stevens-Johnson 综合征病例,其慢性并发症为细支气管炎闭塞。

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication.

机构信息

Department of Pediatrics, Osaka Red Cross Hospital, Osaka, Japan

Department of Pediatrics, Osaka Red Cross Hospital, Osaka, Japan.

出版信息

BMJ Case Rep. 2022 Apr 25;15(4):e249224. doi: 10.1136/bcr-2022-249224.

Abstract

A young girl in her teens presented with fever, rashes and various mucocutaneous symptoms. Flat erythematous macules were seen mainly on the limbs, without blisters or skin detachments. The lips were swollen with crusts and haemorrhage. The oral cavity and pharynx showed ulcerative lesions with exudate. Severe bilateral ocular lesions with pseudomembrane formation and corneal epithelial defects were present. Also, urogenital lesion and gastrointestinal symptoms with frequent haematochezia were observed. Her symptoms and pathological findings were consistent with Stevens-Johnson syndrome. She was treated with prednisolone and methylprednisolone pulse therapy. Her ocular and cutaneous symptoms improved without severe chronic complications. However, 1 month later, she developed dyspnoea, and a pulmonary function test revealed severe obstructive ventilation disorder. After discharge, she was regularly followed up for respiratory complications. High-resolution chest CT performed 9 months after onset revealed mosaic perfusions and bronchiectasis, consistent with bronchiolitis obliterans.

摘要

一位十几岁的年轻女孩出现发热、皮疹和各种黏膜皮肤症状。主要在四肢可见平坦的红斑性斑疹,无水疱或皮肤脱屑。嘴唇肿胀并有结痂和出血。口腔和咽部有渗出性溃疡病变。严重的双侧眼部病变伴有假膜形成和角膜上皮缺损。此外,还观察到泌尿生殖道病变和伴有频繁血便的胃肠道症状。她的症状和病理发现符合史蒂文斯-约翰逊综合征。她接受了泼尼松龙和甲基泼尼松龙脉冲治疗。她的眼部和皮肤症状有所改善,没有出现严重的慢性并发症。然而,1 个月后,她出现呼吸困难,肺功能检查显示严重的阻塞性通气障碍。出院后,她定期接受呼吸并发症的随访。发病后 9 个月进行的高分辨率胸部 CT 显示马赛克灌注和支气管扩张,符合闭塞性细支气管炎。

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