暂时性棘层松解性皮病临床病理观察

Transient acantholytic dermatosis:a clinicopathological analysis

目的通过报道1例暂时性棘层松解性皮病及复习相关文献增加对该病的了解。方法观察患者的临床表现,以石蜡切片行组织形态学观察,并做免疫荧光检测。结果患者男性,88岁。长期卧床,有多种脏器功能损害,且发病前有发热症状。临床以丘疹和水疱为主要皮肤改变。镜下见表皮角质层及颗粒层内有圆体及谷粒,棘细胞空泡变性,棘层松解,形成裂隙和小水疱,伴中性粒细胞浸润,裂隙旁松解的棘层中可见多个微脓疡结构。免疫荧光示IgA、IgM、IgG和C3均为(-)。结论暂时性棘层松解性皮病国内罕见,伴有海绵状水肿的棘层松解是诊断本病的重要线索,结合临床查体和相关病史可以确诊。

Purpose To report a case of transient acantholytic dermatosis with review of the related literatures so as to improve the recognition of this disease.Methods The clinical manifestations of this patient were collected,and the lesion was observed by microscopy.Paraffin-embedded tissue sections were prepared with immunofluorescence.Results A 88-year-old man with long-term in bed,had many functional lesions of organs and had a high body temperature before getting sick.The principal changes of skin were papule and blister,and the microscopic findings were the sphere and grain of epidermis,vacuolar degeneration of acanthocytes,acantholysis,forming a gap and a phlysis with infiltration of some neutrophilic granulocytes,and the microabscess in the surrounding regions of the gap.The staining of immunofluorescence showed that IgA,IgM,IgG and C3 were all negative.Conclusions Transient acantholytic dermatosis is seldomly reported in China.The most important diagnostic clue of this disease is acantholysis with edema like sponge.It is valuable to make a final diagnosis by the combination of physical examinations and correlated clinical history.