帕里 - 罗姆伯格综合征与En Coup De Sabre相关:一个临床病例报告
Gabija Dragunaite1,2, Gabriele Vengalyte3, Jurgita Makstiene4
1Department of Skin and Venereal Diseases, Medical Academy, Lithuanian University of Health Sciences (LSMU), Kaunas, LTU.
Cureus
|December 30, 2025
概括
帕里-罗姆伯格综合征 (PRS) 或渐进性面部血缩,可以发生在成年人. 这一案例表明,PRS从局部性硬皮症演变为局部性硬皮症,强调早期诊断和治疗以获得更好的结果.
科学领域:
- 神经学 神经学
- 皮肤病学 皮肤病学
- 罕见疾病 罕见疾病
背景情况:
- 帕里-罗姆伯格综合征 (PRS) 或渐进性面部血缩是一种罕见的神经皮肤疾病.
- PRS涉及面部组织的渐进,单边缩,通常从童年或成年早期开始.
- 成人发病的PRS病例越来越多地被识别,并与局部性硬化症重叠,特别是en coup de sabre (ECDS) 亚型.
研究的目的:
- 为了呈现一种罕见的与ECDS相关的成人发病PRS病例.
- 要突出诊断的挑战和管理的PRS从成年人局部性硬皮症演变.
主要方法:
- 一个52岁的女性患者的病例报告,患有成人发病的PRS和ECDS.
- 检查患者病史,临床检查,实验室检查,成像,眼科评估和皮肤活检.
- 开始全身的甲状腺素治疗.
主要成果:
- 这位患者呈现出渐进的右半脸缩和额头上线性"剑击"病变,这是ECDS的特征.
- 诊断证实了带有线性硬化症的PRS.
- 甲索开始用于管理功能和美学障碍.
结论:
- 从局部性硬化皮肤症演变为成人发病的PRS需要高度的临床怀疑.
- 早期诊断和及时的免疫抑制治疗对于限制疾病进展和改善PRS患者结果至关重要.
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