Watanabe Shun, Sawa Naoki, Mizuno Hiroki, Hiramatsu Rikako, Hayami Noriko, Yamanouchi Masayuki, Suwabe Tatsuya, Hoshino Junichi, Fujii Takeshi, Hirai Toshihide, Hasegawa Tomoka, Amizuka Norio, Ubara Yoshifumi
Nephrology Center, Toranomon Hospital, Kanagawa, Japan.
Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan.
Bone Rep. 2020 Jul 15;13:100296. doi: 10.1016/j.bonr.2020.100296. eCollection 2020 Dec.
A 56-year-old Japanese woman with a history of palmoplantar pustulosis was admitted for examination due to left femur pain. Radiography and computed tomography showed thickening of the bone on the outer portion of the left femur. Bone scintigraphy of the left femur showed intense radioactive uptake. Consequently, the patient was diagnosed with SAPHO syndrome. Bone histomorphometric analysis of the left femur showed cancellous bone with thickened cortical bone. Whilst normal bone shows cancellous bone with double labeling (normal turn over), and cortical bone with no labeling (low turn over, adynamic state), this case presented with both cancellous and cortical bone with marked double labeling (indicating high turn over), abundant osteoid and woven bone. Immunohistological analysis showed that cells lining the bone surface consisted of osteoblasts and were positive for alkaline phosphatase (ALP). Few to little of these cells were positive for tartrate-resistant acid phosphatase (TRAP)-5B, cathepsin K and matrix metallopeptidase 9 (MMP-9). These results indicate that, in this case study, excessive production of osteoblasts contributed to hyperostosis of the left femur, with abundant osteoid and woven bone. This type of bone formation in SAPHO syndrome is not lamellar bone seen in normal bone, but rather fragile and mechanically weak bone, resulting in bone pain. Doxycycline may be a therapeutic option for bone pain in this patient.
一名56岁有掌跖脓疱病病史的日本女性因左股骨疼痛入院检查。X线摄影和计算机断层扫描显示左股骨外侧骨质增厚。左股骨骨闪烁显像显示放射性摄取增强。因此，该患者被诊断为滑膜炎、痤疮、脓疱病、骨肥厚和骨炎综合征（SAPHO综合征）。左股骨的骨组织形态计量学分析显示松质骨伴有增厚的皮质骨。正常骨的表现为有双重标记的松质骨（正常骨转换）和无标记的皮质骨（低骨转换，静止状态），而该病例的松质骨和皮质骨均有明显的双重标记（表明高骨转换），类骨质和编织骨丰富。免疫组织学分析显示骨表面的细胞由成骨细胞组成，碱性磷酸酶（ALP）呈阳性。这些细胞中很少或几乎没有抗酒石酸酸性磷酸酶（TRAP）-5B、组织蛋白酶K和基质金属蛋白酶9（MMP-9）呈阳性。这些结果表明，在本病例研究中，成骨细胞的过度产生导致了左股骨骨肥厚，伴有丰富的类骨质和编织骨。SAPHO综合征中的这种骨形成不是正常骨中所见的板层骨，而是脆弱且力学强度弱的骨，导致骨痛。强力霉素可能是该患者骨痛的一种治疗选择。
