Hosalkar Rashmi Maruti, Pathak Jigna, Swain Niharika, Mohanty Neeta
Department of Oral Pathology and Microbiology, MGM Dental College and Hospital, Navi Mumbai, Maharashtra, India.
Department of Oral Pathology and Microbiology, Institute of Dental Sciences Siksha O Anusandhan University, Bhubaneswar, Odisha, India.
BMJ Case Rep. 2015 May 12;2015:bcr2014209149. doi: 10.1136/bcr-2014-209149.
Fibrous dysplasia (FD) is a benign skeletal lesion occurring due to mutation of Gs α gene and involves one or multiple bones. We present a case of a 30-year-old female patient, with a 1-year history of swelling under her right eye that had gradually increased in size. Extraoral examination revealed a diffuse swelling extending anteroposteriorly from preauricular region to nasolabial fold, frontonasal region and superoinferiorly from zygoma to body of mandible, causing ipsilateral proptosis and contralateral deviation of nose. Intraoral examination showed obliteration of right upper and lower buccal vestibule. CT disclosed expansile lytic lesions involving multiple skull bones, jaws, sternum, rib and thoracic vertebrae. Histopathology displayed broad and interconnected trabeculae connected to the host bone exhibiting reversal lines resembling the mosaic pattern of Paget's disease. These features suggested pagetoid polyostotic FD. The patient underwent cosmetic recontouring and is under regular follow-up postoperatively.
骨纤维异常增殖症（FD）是一种由于Gsα基因突变导致的良性骨骼病变，可累及一块或多块骨骼。我们报告一例30岁女性患者，其右眼下方肿胀1年，且逐渐增大。口外检查发现弥漫性肿胀，从前耳区域向后延伸至鼻唇沟、额鼻区域，从颧骨向下至上颌骨体部，导致同侧眼球突出和对侧鼻偏斜。口内检查显示右上颌和下颌颊侧前庭消失。CT显示多个颅骨、颌骨、胸骨、肋骨和胸椎出现膨胀性溶骨性病变。组织病理学显示宽阔且相互连接的骨小梁与宿主骨相连，呈现出类似佩吉特病镶嵌图案的反转线。这些特征提示为变形性多骨型骨纤维异常增殖症。该患者接受了美容整形修复，术后定期随访。
