SAPHO综合征表现为急性滑膜炎和左声带麻痹:一个具有挑战性的诊断。

IF 1.9 Q2 ORTHOPEDICS Clinical Medicine Insights. Arthritis and Musculoskeletal Disorders Pub Date : 2022-09-07 eCollection Date: 2022-01-01 DOI:10.1177/11795441221118918
Beatrice Maranini, Giovanni Ciancio, Rosa Rinaldi, Massimo Borrelli, Maura Pugliatti, Marcello Govoni
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引用次数: 0

摘要

SAPHO(滑膜炎、痤疮、脓疱病、骨质增生和骨炎)是一种罕见的综合征,主要以皮肤和骨关节表现为特征。最典型的骨关节表现局限于前胸壁,通常包括非感染性骨炎、骨质增生和胸锁关节滑膜炎。然而,SAPHO综合征的临床表现可能是相当异质的。一些临床和放射学特征与其他明确的病理实体相同,临床体征和症状经常出现在不同的时间点。主要由于SAPHO综合征的复杂性和罕见性,目前尚无有效的SAPHO综合征诊断标准。骨骼周围软组织的炎症和可能的神经压迫可导致吞咽困难、声音减退或气道阻塞。因此,神经系统的表现可能是这种多器官受累的一部分。在这里,我们报告一个SAPHO综合征的病例,其发病症状不典型,其特征是左声带麻痹,急性颈部疼痛,由溶骨性寰枢椎病变引起,并有不寻常的皮肤表现,诊断为真皮中弹性松解。据我们所知,最近的两种是在SAPHO综合征病例中首次描述的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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SAPHO Syndrome Presenting With Atlo-Epistrophic Synovitis and Left Vocal Cord Paresis: A Challenging Diagnosis.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare syndrome mainly characterized by cutaneous and osteoarticular manifestations. The most typical osteoarticular manifestations are localized to the anterior chest wall and include a usually noninfectious osteitis, hyperostosis, and synovitis of the sternoclavicular joints. However, clinical presentation of SAPHO syndrome can be quite heterogeneous. Several clinical and radiological features are shared with other well-defined pathological entities, and clinical signs and symptoms often occur at different timepoints. Mainly due to this complexity and its rarity, there are currently no validated diagnostic criteria for SAPHO syndrome. Inflammation of the soft tissues around the bones and possible nerve compression could contribute to dysphagia, hypophonia, or obstruction of the airways. Neurologic manifestations could therefore be part of this multiorgan involvement. Here, we present a case of SAPHO syndrome with atypical onset symptoms, characterized by left vocal cord paralysis, acute neck pain due to osteolytic atlantoepistrophic lesion, and an unusual cutaneous manifestation, diagnosed as mid-dermal elastolysis. The latest two, to the best of our knowledge, have been here first described in a case of SAPHO syndrome.

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CiteScore
4.40
自引率
0.00%
发文量
14
审稿时长
8 weeks
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