摘要
目的:提高对复发性线状棘层松解性皮病的认识,探讨其诊断依据和有效的治疗方法。方法:就1例复发性线状棘层松解性皮病患者及相关文献进行临床分析,在病因、诊断及治疗等方面对本病进行讨论分析。结果:本例为6个月大男性患儿,右臀部至腘窝,右肘关节伸侧以及右胸腹部见沿Blas- chko线分布的暗红斑、水疱、糜烂和浅溃疡,组织病理学改变同Hailey-Hailey病(家族性慢性良性天疱疮),经适量皮质类固醇激素及敏感抗生素治疗后缓解;本病自1985年首例报道以来,至今国内外仅有5例报告,皮损均具有沿Blaschko线呈单侧分布的特征。结论:复发性线状棘层松解性皮病是一种极为罕见的、反复发作的痣样疾病,皮疹以沿Blaschko线分布伴有水疱和糜烂的红色斑块为特征,组织病理具有不完全棘层松解,倒塌的砖墙样改变,与Hailey-Hailey病不能区别,两者的诊断主要依据其特征性的临床表现。
Objective： To get inside view of relapsing linear acantholytic dermatosis and explore the diagnostic evidence as well as effective therapeutic methods. Methods：Analyze and discuss the etiology, diagnosis and treatment of the disease ,from clinical analysis of one case of relapsing linear acantholytic dermatosis and related literatures. Results： This report describes a six -month -old boy, exhibited lesions along with Blaschko Line. The lesions were characterized by recurrent erythema, vesicula eruptions, erosions, superficial ulcers, effusions and incrustation on the right haunch, the flexor aspect of the fight thigh, the extensor aspect of the right elbow joint, and the right chest and abdomen wall, and were relieved after treatment with corticosteroid and sensitive antibiotics. The histopathological feature was the same as Hailey - Hailey disease. There were only 5 cases of this disease reported since the first case reported in 1985 over the world. Conclusion： Relapsing linear acantholytic dermatosis is a extremely rare recureds nevoid illness, the lesions of which distribute along Blaschko line accompanied by vesicles and red plaques with erosions. Histopathology of a representative lesion revealed not entirely acantholysis with the collapse of the brick wall-like change, consistent with features of Hailey - Hailey disease. These two diseases are definitely diagnosed mostly depending on its specific clinical manifestations.
出处
《岭南皮肤性病科杂志》
2007年第5期280-283,共4页
Southern China Journal of Dermato-Venereology
