汗孔角化症64例临床与病理分析

Clinical and Pathological Analysis of 64 Cases of Porokeratosis

目的探讨汗孔角化症(porokeratosis,PK)的临床和组织病理特征,特别是生殖器区域汗孔角化症。方法回顾性分析64例PK患者的临床及组织病理资料。结果64例患者中,男46例,女18例,平均发病年龄36.2岁(2~73岁),平均病程9.4年(1个月~60余年)。皮损累及部位:面部受累9例,躯干受累23例,上肢受累26例,下肢受累34例,生殖器及邻近区域受累14例,其他部位受累6例。PK组织病理改变有其特异性,均可见角化不全柱,其下方部分颗粒层减少或消失,可见角化不良细胞。生殖器区域PK临床表现呈多样化改变,组织病理上常可见多发性角化不全柱。结论PK可累及任何部位,临床表现差异较大,特别是生殖器区域PK极易被误诊误治,组织病理学检查在PK的诊断中具有重要作用,有利于指导临床诊治。

Objective To study the clinical and histopathological features of porokeratosis,especially the genital porokeratosis.Methods The clinical and histopathological data of 64 cases of porokeratosis were analyzed retrospectively.Results The male patients were 46 cases(71.86%)and the females were 18 cases(28.13%).The average age of onset was 36.2 years(2~73).The average disease duration was 9.4 years(1 month to 60 years).It affected almost any areas of the body which include face(9 cases),trunk(23 cases),upper extremities(26 cases),lower extremities(34 cases),genital and adjacent areas(14 cases),and other areas(6 cases).Pathological changes of porokeratosis were relatively specific,which includes cornoid lamella with underlying partial deceased or totally disappeared granular layer and dyskeratotic cells.The clinical manifestations of genital porokeratosis are varied,and multiple contiguous cornoid lamellae in the epidermis could often be observed in histopathology.Conclusion Porokeratosis can affect almost any areas of the body,and the clinical manifestations vary greatly.Genital porokeratosis is easily to be misdiagnosed and mistreated.Histopathology plays an important role in the diagnosis and treatment for porokeratosis.