PDF(Pubmed)
Abstract：
Scleredema is a rare sclerotic skin disorder that typically develops in conjunction with diabetes, monoclonal gammopathy, or infection and commonly involves the neck, back, trunk, and arms. Scleredema can be categorized into three types according to its cause. The local examination of the lesion is characterized by non-pitting induration that follows a symmetrical spread with sparing of the hands and feet. We present a case of a 19-year-old female patient that presented to the outpatient clinic complaining of facial skin lesions that have been progressive for four years. The skin lesions were found to involve the neck, back, trunk and both arms sparing the hands and there was no systemic involvement of the disease. The patient is non-diabetic but reported frequent attacks of tonsillitis over the past months. Initially, punch biopsy showed no signs of scleredema; however, repeated biopsy at follow-up confirmed the presence of mucin deposits which are suggestive features of scleredema. Due to the similarities with various other diseases, the diagnosis requires clinical and histopathological exclusion which makes the diagnosis quite challenging. It almost always starts in the neck but can present initially in rare cases from the face spreading symmetrically. Close follow-up and continuous monitoring are necessary for systemic organ involvement.
摘要：
Scleedema是一种罕见的硬化性皮肤病，通常与糖尿病一起发展，单克隆丙种球蛋白病，或感染，通常累及颈部，回来,树干,和手臂。根据其原因，Scleedema可以分为三种类型。病变的局部检查的特征在于非点蚀性硬结，随后对称扩散，同时保留手和脚。我们介绍了一例19岁的女性患者，该患者出现在门诊诊所，抱怨面部皮肤病变已经进展了四年。发现皮肤损伤累及颈部，回来,躯干和双臂保持双手，没有全身性疾病的参与。该患者是非糖尿病患者，但报告在过去几个月中经常发作扁桃体炎。最初，穿刺活检未显示硬肿症的迹象；然而，随访时重复活检证实存在粘蛋白沉积，这提示了硬肿症的特征。由于与其他各种疾病的相似性，诊断需要临床和组织病理学排除，这使得诊断相当具有挑战性。它几乎总是从颈部开始，但在极少数情况下可以从面部对称扩散开始出现。对于全身器官受累，密切随访和持续监测是必要的。
