22例汗孔角化症的临床研究

Clinical Study on 22 Cases of Porokeratosis of Mibell

通过对22例汗孔角化症患者的发病特点、临床表现、并发症、临床治疗以及转归等进行回顾性分析和研究,全面认识汗孔角化症。在我院从2007年12月至2013年07月期间治疗的汗孔角化症患者中,详细询问家族史、病史、发病情况及提供伴发病诊断依据和治疗情况,体格检查及实验室检查,辨别特征性皮损,对其进行病理活检确诊,并进行相应治疗。有22例皮损形态、组织病理改变均具有特征性,确诊为汗孔角化症,19例患者为青年人,其中16例有家族遗传史,19例无系统性疾病史;3例老年患者,无家族遗传史,其中2例患糖尿病和高血压多年,常年服药;另外1例有骨髓移植史。汗孔角化症是显性遗传病,特征性皮损为棕褐色斑块,中心萎缩,境界清楚的角化性边缘,一般经临床表现及病理检查确诊。一直以来认为家族遗传史、光照、外伤、感染因素最易诱发此病,但近年来随着器官移植、免疫抑制剂的应用、机体免疫力下降等免疫机制紊乱因素成为汗孔角化症的发病又一重要原因。

To fully understand this disease through retrospective analysis on characteristic, clinical manifestation; complication; clinical remedy and turnover of 22 Cases of Porokeratosis of Mibell. The patients from December 2007 to July 2013 were included. They were treated and diagnosed by pathological inspecting. 22 patients that Cutaneous lesions and histopathologyical changes are distinctive are diagnosed Porokeratosis, and 19 patients are younger, 16 patients have similarly familial history cases, 19 patients have no systemic disease; Two elderly patients with type 2 diabetes mellitus and hypertension treated with medicine long years have not similarly familial history cases;Another one with myelodysplatic syndrome underwent bone marrow transplantation. According to the genetic analysis, this disease is caused by dominant inheritance. Brown plaques are characteristic for dermatology lesions; the patches were confluent with an atrophic center and a well-demarcated keratotic border. It is diagnosed for clinical manifestation and pathology. Formerly familial heredity, sunlight, trauma and infection, which were lead to porokeratosis easily. But recently, with the widespread use of organ transplantation, immunosuppressive treatments and lowering of immunocompetence, mechanism of immune disorder is playing another significant role in this disease onset.