Cutaneous Manifestations of Scleroderma: A Case Report

IF 0.2 Q4 PHARMACOLOGY & PHARMACY
Tessa Thendria, Amira Suryani Rahmatika, Evy Ervianti, Sawitri Dwi, Murtiastutik Trisiswati, Indranarum Hasnikmah, Mappamasing Septiana, Widyantari
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Abstract

Background: Scleroderma or systemic sclerosis is a rare multisystemic autoimmune disease characterized by vasculopathy, inflammation, and progressive fibrosis of the skin and multiple organs. Cutaneous manifestations and Raynaud’s phenomenon, usually becomes the initial presentation of scleroderma which noticed by the patient. Case: A 47-year-old-women presented with hardened and thickened skin on her arms and legs for 3 years which spreaded to her face and trunk. The lesions initially appeared as multiple red patches which progressed into white, thick and hard patches. The patient had history of recurrent Raynaud's phenomenon, dry cough, and shortness of breath. In the physical examination, the patient had skin hardening with salt and pepper appearance on her upper back and chest. The thoracic CT scan revealed interstitial lung disease. The patient diagnosed as definitive scleroderma based on The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria with score of 18. She has treated with methotrexate, methylprednisolone, topical corticosteroid and moisturizer. The skin lesion improved with the reduction of Rodnan skin score from 31 to 19 after 4 months of treatment. Conclusion:  Cutaneous manifestation  could  be  the  early  alarm  and  initial  manifestation  of scleroderma.  As the pathology  is deep beyond  the skin,  throughout examination  should  be performed to find any organ involvement. Dermatologist play a significant role in early identification of skin disorder and made multidiciplinary refferal. Early diagnosis and prompt treatment could reduce morbidity and mortality in scleroderma
硬皮病的皮肤表现1例
背景:硬皮病或系统性硬化症是一种罕见的多系统自身免疫性疾病,其特征是皮肤和多器官的血管病变、炎症和进行性纤维化。皮肤表现和雷诺现象,通常成为硬皮病患者注意到的最初表现。病例:一名47岁女性,手臂和腿部皮肤硬化和增厚3年,并蔓延至面部和躯干。病变最初表现为多个红色斑块,逐渐发展为白色,厚而硬的斑块。患者有反复出现雷诺氏现象、干咳、呼吸短促等病史。查体时,患者上背部及胸部皮肤硬化,呈盐椒样。胸部CT扫描显示肺间质性疾病。根据欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)标准,患者被诊断为明确的硬皮病,评分为18分。她接受了甲氨蝶呤、甲基强的松龙、局部皮质类固醇和保湿霜的治疗。治疗4个月后,罗德曼皮肤评分从31分降至19分,皮肤病变得到改善。结论:皮肤表现可能是硬皮病的早期报警和初始表现。由于病理深度超出皮肤,应进行全面检查以发现任何器官受累。皮肤科医生在皮肤病的早期识别中发挥着重要作用,并进行了多学科转诊。早期诊断和及时治疗可降低硬皮病的发病率和死亡率
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