Proteus综合征2例临床病理分析

发布时间：2018-03-17 09:01

本文选题：变形综合征　切入点：脑回状纤维组织增生　出处：《临床与实验病理学杂志》2016年07期 　论文类型：期刊论文

【摘要】：目的探讨Proteus综合征(Proteus syndrome,PS)的临床病理特点及鉴别诊断。方法回顾性分析2例具有脑回状纤维组织增生特征的PS临床表现与组织病理学特点,并复习相关文献。结果患者均自幼发病,随年龄增长进行性加重,例1表现为双侧手指和足趾甲床不对称性脑回状纤维组织增生以及多发性色素痣,例2表现为左侧下肢骨骼、左足、左足趾及软组织不对称性过度增生肥大,足趾软组织脑回状纤维组织增生,左下肢及足背血管畸形。病理组织学表现为真皮内大量胶原纤维增生、成纤维细胞稀疏、弹力纤维缺乏等病理学特点。结论 PS是一种罕见的先天性错构瘤,具有多变的畸形特征,其正确诊断需与临床表现密切结合,并与其表现相似的先天性综合征鉴别。
[Abstract]:Objective to investigate the clinicopathological features and differential diagnosis of Proteus syndrome (Proteus syndromes). Methods the clinical and histopathological features of 2 cases of PS with the characteristics of gyrus fibrous hyperplasia were retrospectively analyzed. Results all the patients developed since childhood and were aggravated with age. Case 1 presented bilateral finger and toe nail bed asymmetrical ileal fibrous hyperplasia and multiple pigmented nevus, case 2 showed left lower limb bone. Left foot, left toe and soft tissue hypertrophy, left toe soft tissue gyrus fibrous tissue hyperplasia, left lower extremity and dorsal foot vascular malformation. Conclusion PS is a rare congenital hamartoma with variable deformities. The correct diagnosis of PS should be closely combined with clinical manifestations and differentiated from congenital syndromes with similar manifestations.
【作者单位】：中山大学东莞东华医院病理科;
【分类号】：R597

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