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青少年 SAPHO 综合征:一例具有非典型严重全身影响的临床病例。

SAPHO syndrome in an adolescent: a clinical case with unusual severe systemic impact.

机构信息

Adolescent Medicine Clinic, Department of Pediatrics, Hospital de Santa Maria, Lisbon, Portugal.

Adolescent Medicine Clinic, Department of Pediatrics, Hospital de Santa Maria, Lisbon, Portugal; Rheumatology Research Unit, Molecular Medicine Institute, Faculty of Medicine, Lisbon University, Lisbon, Portugal.

出版信息

J Adolesc Health. 2014 Aug;55(2):304-6. doi: 10.1016/j.jadohealth.2014.02.004. Epub 2014 Apr 13.

Abstract

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes both dermatological and rheumatologic symptoms. Being a rare condition, the diagnosis is frequently late. The authors report a case of a 13-year-old boy diagnosed with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome with unusual severe systemic repercussions. The patient presented with acne conglobata, inability to walk due to pain and weakness and weight loss. Bone scintigraphy was suggestive of sacroiliitis, and lumbar spine x-ray showed signs of hyperostosis. His clinical state improved after treatment with nonsteroidal anti-inflammatory drugs, methotrexate, clindamycin, and isotretinoin. A review of the clinical aspects of this syndrome is presented, emphasizing how this underdiagnosed syndrome can lead to severe weight loss and significant functional and psychological impairment at an early age.

摘要

SAPHO(滑膜炎、痤疮、脓疱病、骨肥厚和骨炎)综合征包括皮肤科和风湿科的症状。由于是一种罕见的疾病,其诊断常常较晚。作者报告了一例 13 岁男孩患有滑膜炎、痤疮、脓疱病、骨肥厚和骨炎综合征,伴有不寻常的严重全身并发症。该患者表现为聚合性痤疮,因疼痛、虚弱和体重减轻而无法行走。骨闪烁显像提示为骶髂关节炎,腰椎 X 光显示骨肥厚的迹象。他的临床状况在接受非甾体抗炎药、甲氨蝶呤、克林霉素和异维 A 酸治疗后得到改善。本文介绍了该综合征的临床方面,强调了这种诊断不足的综合征如何导致在早期出现严重的体重减轻以及显著的功能和心理障碍。

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