Limited scleroderma - a case report

S. Knežević, S. Djordjevic
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Abstract

Introduction. Systemic sclerosis is a rare autoimmune disorder of the connective tissue, gastrointestinal tract, lungs, kidneys, and musculoskeletal tissue. It predominantly affects women. The localized variant is limited scleroderma. Case Report. We present a 64-year-old female patient with the diagnosis of limited scleroderma that has lasted for thirteen years. She had hyperpigmentation, telangiectasias, and progressive skin tightening of the face and fingers. Her blood test was positive for antinuclear antibodies. Sclerodactyly began in the distal phalanx. Tender and painful calcium deposits appeared subcutaneously on the surface of palms and knees, radiographically confirmed. The patient was treated with surgical debridement, vasodilating agents, corticosteroids, diltiazem, sildenafil, nitro paste, antiplatelet drugs, and physical therapy. Conclusion. It is necessary to control numerous factors that affect daily functioning, including nutrition, pain therapy, musculoskeletal dysfunctions, and emotional and social aspects caused by deformities. Targeted therapy in the early stages of the disease, before irreversible damage occurs, improves the overall quality of life.
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局限性硬皮病1例报告
介绍。系统性硬化症是一种罕见的自身免疫性疾病,涉及结缔组织、胃肠道、肺、肾脏和肌肉骨骼组织。它主要影响女性。局部变异为局限性硬皮病。病例报告。我们报告一位64岁的女性患者,诊断为局限性硬皮病,持续了13年。她有色素沉着,毛细血管扩张,面部和手指皮肤逐渐收紧。她的血检抗核抗体呈阳性。硬指始于远端指骨。手掌和膝盖皮下出现疼痛的钙沉积,x线片证实。患者接受手术清创、血管舒张剂、皮质类固醇、地尔硫卓、西地那非、硝基糊、抗血小板药物和物理治疗。结论。有必要控制许多影响日常功能的因素,包括营养、疼痛治疗、肌肉骨骼功能障碍以及由畸形引起的情感和社会方面。在疾病的早期阶段,在不可逆转的损害发生之前进行靶向治疗,可以提高整体生活质量。
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