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临床少见的皮肤僵硬综合征

         

摘要

Objective To investigate the clinical characteristics of skin syndrome Stiff (SSS) in order to reduce the misdiagnosis and mistreatment rates. Methods A case of SSS in our hospital was analyzed retrospectively and the literature was reviewed. Results The patient had hardened skin on the right side of his body for 4 years, increased body hair for 3 years, limited neck activity for 1 year, and had been misdiagnosed as having scleroderma in other hospitals, and undergone corresponding treatment with no obvious improvement. The diagnosis of SSS was therefore made based on its typical clinical manifestation ( early childhood onset, skin lesions like stone, but the appearance and texture of the skin were normal) and al-cian blue staining on histological examination. There were no effective treatment options for SSS. The patient was advised to have physical rehabilitation, and was followed-up for 3 months, but the skin stiffness degree had no obvious improvement. The patient is still in further follow-up. Conclusion SSS is an extremely rare disease,and is easy to be misdiagnosed as scleroder-ma. To raise awareness and alertness are essential in reducing misdiagnosis and mistreatment rates.%目的 探讨皮肤僵硬综合征(stiff skin syndrome,SSS)的临床特点,以减少误诊误治.方法 对SSS 1例的临床资料进行回顾性分析并复习相关文献.结果 本例因右半身皮肤变硬4年,体毛增多3年,颈部活动受限1年曾在多家医院就诊,均诊断为硬皮病并予相应治疗,未见明显好转.后就诊我院依据临床表现(幼儿期发病,皮损呈石头状,但外观和皮肤纹理正常)及皮肤活检示阿新兰染色附属器周围阳性诊断为SSS.该病无特效治疗方法 ,建议患者积极功能锻炼,随访3个月,皮肤僵硬程度无明显改变,现仍在随访中.结论 SSS临床少见,容易与硬皮病混淆而误诊,对本病提高认识和警惕是避免或减少误诊误治的关键.

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