Abstract：
Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disease characterized by bone inflammation and skin manifestations including acne, palmoplantar pustulosis, psoriasis, or hidradenitis suppurativa. SAPHO syndrome is considered on the same spectrum as chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis (CNO/CRMO), the former often being the nomenclature in adults and the latter in children. The diagnosis is made on patterns of clinical manifestations and is a diagnosis of exclusion. While skin and bone manifestations are commonly described with SAPHO syndrome, pleural involvement is rare, and few cases have been described in the literature, especially in pediatric patients. Herein we present a 14-year-old female with a past medical history of hidradenitis supprtiva, eczema, psoriasis, and a prior episode of culture-negative osteomyelitis who presented to the emergency room with chief complaints of right sided pain with inspiration and back pain. Exam revealed palmoplantar pustulosis, hidradenitis supprativa, psoriasis, and tenderness of vertebrae. Imaging showed a right sided pleural effusion and multiple sites of osteitis. Laboratory evaluation revealed elevated inflammatory markers, an exudative pleural effusion with neutrophilic predominance, and no evidence of malignancy, infection, or immunodeficiency. The patient was diagnosed with SAPHO syndrome and treated with naproxen, methotrexate, and golimumab with significant improvement including resolution of the pleural effusion. Pediatric SAPHO syndrome is a rare disease that classically causes osteitis and skin manifestations. This case highlights that pleural effusion can be a rare manifestation of pediatric SAPHO syndrome. Patients with suspected SAPHO syndrome with respiratory symptoms should be evaluated for pleural effusion.
摘要：
滑膜炎,痤疮,脓疱病,骨增生,骨炎（SAPHO）综合征是一种罕见的自身炎症性疾病，其特征是骨炎症和皮肤表现，包括痤疮，掌plant脓疱病，牛皮癣,或化脓性汗腺炎。SAPHO综合征与慢性非细菌性骨髓炎/慢性复发性多灶性骨髓炎（CNO/CRMO）相同，前者通常是成人的命名法，后者是儿童的命名法。诊断是根据临床表现的模式进行的，是排除的诊断。虽然皮肤和骨骼表现通常描述为SAPHO综合征，胸膜受累是罕见的，文献中很少描述案例，尤其是儿科患者。在这里，我们介绍了一名14岁的女性，其既往病史为汗腺炎，湿疹,牛皮癣,和之前的文化阴性骨髓炎发作，他出现在急诊室，主要主诉右侧疼痛伴吸气和背部疼痛。检查显示掌plant脓疱病，超级汗腺炎,牛皮癣,和椎骨的压痛。影像学显示右侧胸腔积液和多个骨炎部位。实验室评估显示炎症标志物升高，以嗜中性粒细胞为主的渗出性胸腔积液，没有恶性肿瘤的证据,感染,或免疫缺陷。患者被诊断为SAPHO综合征，并接受萘普生治疗，甲氨蝶呤,和戈利木单抗有显著改善，包括胸腔积液的消退。小儿SAPHO综合征是一种罕见的疾病，通常会引起骨炎和皮肤表现。该病例强调胸腔积液可能是小儿SAPHO综合征的罕见表现。怀疑有呼吸道症状的SAPHO综合征患者应进行胸腔积液评估。
