获得反应性穿孔性原体 - - 在常见疾病中发生的罕见实体:系统性审查和我们的个人经验
Maria Alexandra Junghetu1,2, Cristina Violeta Tutunaru3, Simona Laura Ianoși3
1Department of Oncologic Dermatology, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
Journal of clinical medicine
|January 10, 2026
概括
获得的反应性穿孔性原蛋白症 (ARPC) 是一种罕见的皮肤疾病,通常与全身性疾病有关. 本综述总结了ARPC诊断,管理,并强调了临床试验的必要性.
科学领域:
- 皮肤病学 皮肤病学
- 病理学 病理学 病理学
- 系统性疾病 系统性疾病
背景情况:
- 获得的反应性穿孔性原蛋白症 (ARPC) 是一种罕见的皮肤疾病.
- 它通常影响患有糖尿病,慢性病 (CKD),心血管问题和癌症等并发症的成年人.
- 药物诱导和创伤相关的病例也被记录在案.
研究的目的:
- 系统地审查目前ARPC的诊断和治疗策略.
- 为管理这种罕见疾病提供最新的见解.
- 报告与CKD相关的ARPC病例.
主要方法:
- 系统的文献审查遵循PRISMA 2020指南.
- 在PubMed数据库中搜索过去15年内公开访问的初级人体研究.
- 包括已成年患者在内,其组织病理学证实了ARPC.
主要成果:
- 分析了27项研究 (病例报告/系列),患者平均年龄为60.8岁.
- 大多数ARPC病例呈现出并发症;药物诱导的病例也被注意到.
- 诊断线索包括状斑块与中央角质塞,支持皮肤镜,反射共聚焦显微镜,和强制性组织病理学显示跨皮肤原蛋白消除.
结论:
- 诊断ARPC需要对潜在的系统性疾病进行全面的检查,包括恶性瘤.
- 目前的治疗方法包括抗组胺剂,溶剂,皮质类固醇,视网类固醇,光疗,dupilumab和allopurinol.
- 需要进行进一步的临床试验,以建立ARPC的最佳治疗方案.
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