Additional skeletal radiographs are shown below. Click to enlarge.
2.What is the most likely diagnosis?
最有可能的诊断是什么?
Gout 痛风
Calcific bursitis 钙化性滑囊炎
Calcific myositis 钙化性肌炎
Scleroderma 硬皮病
Old frostbite 陈旧性冻伤
选择题答案:
Pigmented villonodular synovitis (PVNS)
Scleroderma
Findings and Diagnosis
FindingsThe left foot has rounded and amorphous calcifications in the plantar soft tissues at the level of the fifth metatarsophalangeal (MTP) joint. Hands show soft-tissue calcifications with erosive changes of the tufts of the distal phalanges of the thumb and index fingers of the left hand. There is joint space narrowing of the interphalangeal joints. Elbow shows calcifications in the soft tissues of the posteromedial right elbow.
左足底第五跖趾关节处、软组织内可见多发类圆形钙化灶。
双手软组织多发钙化,左手拇指、食指远端指骨骨质略受侵。指间关节间隙狭窄。
肘关节后内侧软组织内可见钙化灶。
Differential Diagnosis
Scleroderma 硬皮病
Crystalline arthropathy (gout or calcium pyrophosphate deposition disease)晶体性关节病(痛风或焦磷酸盐沉积钙化)
Calcific bursitis 钙化性滑囊炎
Myositis (trauma) 创伤性肌炎
Renal osteodystrophy 肾病性骨营养不良
DiagnosisScleroderma
硬皮病
Discussion
Systemic sclerosis (the umbrella term encompassing scleroderma) is a disease caused by inflammation with subsequent excessive collagen types 1 and 3 deposition with resultant fibrosis and microvascular occlusion. The prevalence is approximately 240 cases per million population with an average life expectancy of 12 years from diagnosis. Black people (particularly young, black women) are more commonly affected. Involvement of the skin and soft tissues is often most readily apparent, although the disease affects many internal organs and vascular structures.系统性硬化症(包括硬皮病)是指炎症引起胶原蛋白I和III过量沉积,最终导致纤维化和微血管栓塞。该病的发病率约240/1,000,000,确诊该病后的平均生存时间约12年。
黑人(特别是年轻的黑人女性)好发。该病可累及全身多个脏器及血管,但最多发生于皮肤和软组织。
Radiologic overviewWith regard to musculoskeletal imaging, the most common findings of scleroderma include dystrophic soft-tissue calcifications as seen in this case. Acro-osteolysis (resorption of the tuft of the distal phalanx) can occur due to tight atrophic skin. There is often soft-tissue atrophy and erosive changes of the distal and proximal interphalangeal joints.
Other common radiologic findings in scleroderma include pulmonary fibrosis and dilation and dysmotility of the gastrointestinal tract (most commonly affecting the esophagus).
硬皮病其他常见的影像学表现包括:肺纤维化、消化道蠕动异常(最常见于食管)。
Key Points
Scleroderma is a progressive disease resulting in fibrosis of any organ system.
硬皮病是一种渐进性疾病,可累及全身任何器官,引起器官的纤维化。
Findings include soft-tissue calcification, acro-osteolysis, skin atrophy, and interphalangeal joint erosions.主要表现包括软组织钙化、肢端骨质溶解、皮肤萎缩、指间关节受侵。
If the diagnosis is considered, search for findings in other examinations for supportive evidence.如果怀疑硬皮病,可以检查其他部位,发现其他组织器官的异常以支持诊断。