Jecan Cristian Radu, Bedereag Ştefan Iulian, Sinescu Ruxandra Diana, Grigorean Valentin Titus, Cozma Cristina Nicoleta, Bordianu Anca, Florescu Ioan Petre
Department of Plastic and Reconstructive Surgery, "Prof. Dr. Agrippa Ionescu" Emergency Clinical Hospital, Bucharest, Romania;
Rom J Morphol Embryol. 2016;57(2 Suppl):865-869.
Calcinosis consists of abnormal calcium deposition in soft tissues, which appears often in patients with limited systemic sclerosis, being one of the criteria of CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. With a long evolution, the aim of the treatment is to control the symptoms and prevent complications. In this article, we present the challenging management of a profuse lesion of calcinosis in a patient with systemic sclerosis. We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all joints. The interscapular vertebral tumoral mass was excised and the defect was closed. Histopathological examination revealed cutaneous calcinosis, probably associated with CREST syndrome, a type of scleroderma. Postoperative results were favorable and no local complications were encountered. Six months follow-up revealed no evidence of recurrence. Despite the size and the invasion of the tumor in the muscle, complete resection was possible with an adequate reconstruction; the postoperative result being acceptable. With a lower response to medication, surgical treatment is considered the only option for treating symptomatic lesions of calcinosis in order to improve quality of life.
钙质沉着症是指软组织中出现异常钙沉积,常见于局限性系统性硬化症患者,是CREST(皮肤钙质沉着、雷诺现象、食管功能障碍、指端硬化和毛细血管扩张)综合征的诊断标准之一。随着病情的长期发展,治疗目的是控制症状并预防并发症。在本文中,我们介绍了一位系统性硬化症患者钙质沉着症大量病变的挑战性治疗过程。我们描述了一名52岁患有系统性硬化症和CREST综合征的女性病例,她因几乎所有关节处出现多个疼痛且致残的肿瘤性肿块而入住我们科室。切除了肩胛间椎体肿瘤性肿块并封闭了缺损处。组织病理学检查显示为皮肤钙质沉着症,可能与CREST综合征(一种硬皮病类型)相关。术后结果良好,未出现局部并发症。六个月的随访未发现复发迹象。尽管肿瘤体积较大且侵犯了肌肉,但通过充分的重建仍可实现完整切除;术后结果可以接受。由于药物治疗反应较低,手术治疗被认为是治疗有症状的钙质沉着症病变以改善生活质量的唯一选择。