Boussoffara L, Fekih L, Fenniche S, Farhati S, Hassène H, Ben Abdelghaffar H, Oueslati I, Belhabib D, Ben Miled K, Lamine Megdiche M
Service de pneumologie Ibn Nafiss. Hôpital Abderrahmen Mami Ariana. Tunisie.
Rev Mal Respir. 2009 Nov;26(9):1007-9. doi: 10.1016/s0761-8425(09)73339-x.
Osteopoikilosis is a rare, inherited and usually asymptomatic sclerosing bone dysplasia of unknown etiology which predominantly involves the appendicular and rarely the axial skeleton.
We report the case of a 24 year old man who was hospitalized for pleural empyema and treated with antibiotics for six weeks in addition to pleural evacuation and physiotherapy. The diagnosis of osteopoikilosis associated with the pleural empyema was made on the radiological findings. In fact the chest X-Ray showed spherical areas of increased bone density in both humeral epiphyses. In order to explore these bone abnormalities further investigations were performed, including red and white blood cell counts, sedimentation rate and protein electrophoresis. There were no biological abnormalities. Radiography of the whole skeleton showed disseminated sclerotic lesions in the pelvis and the metacarpal and carpal bones of both hands. A neoplastic aetiology was excluded. In the light of these investigations, the diagnosis of osteopoikilosis was established.
Widespread osteopoikilosis can be revealed on chest radiography.
骨斑点症是一种罕见的、遗传性的、通常无症状的硬化性骨发育异常疾病,病因不明,主要累及四肢骨骼,很少累及中轴骨骼。
我们报告一例24岁男性患者,因胸腔积脓住院,除胸腔引流和物理治疗外,接受了六周的抗生素治疗。根据影像学检查结果诊断为与胸腔积脓相关的骨斑点症。实际上,胸部X线片显示双侧肱骨骨骺有球形骨密度增高区。为进一步探究这些骨骼异常情况,进行了包括红细胞和白细胞计数、血沉及蛋白电泳在内的进一步检查。未发现生物学异常。全身骨骼X线检查显示骨盆及双手掌骨和腕骨有弥漫性硬化性病变。排除了肿瘤病因。根据这些检查,确诊为骨斑点症。
胸部X线检查可发现广泛的骨斑点症。