摘要
目的分析线状IgA大疱性皮病的临床特点、治疗及预后,提高临床医生对本病的认识。方法回顾性分析本科2004年1月-2011年12月诊治的15例线状IgA大疱性皮病的临床资料。结果男6例,女9例;儿童型5例,成人型10例;皮损初始部位:面部4例,口腔黏膜及双下肢各3例;累及躯干和四肢12例,14例临床表现为红斑或正常皮肤上出现水疱,6例水疱呈环形或半环形排列。皮损组织病理均见表皮及复层鳞状上皮下水疱或裂隙,直接免疫荧光均表现为基底膜带线状IgA沉积。13例予糖皮质激素治疗,皮损均控制;2例予氨苯砜治疗,均发生不良反应。结论线状IgA大疱性皮病的皮损以口腔黏膜、面部及下肢首发居多,好发于躯干和四肢,临床表现为红斑或正常皮肤上出现水疱,呈孤立、环形和/或半环形排列;组织病理可见表皮或复层鳞状上皮下水疱或裂隙,直接免疫荧光示基底膜带线状IgA沉积是诊断的主要依据。治疗上可选用氨苯砜、糖皮质激素和磺胺类药物等。
Objective To analyze the clinical features of linear IgA bullous dermatosis （LABD） and have a better understanding of this disease to clinicians. Methods Clinical data of 15 cases with LABD were analyzed retrospectively. Results Among 15 cases, 6 were males and 9 were females, 10 were adults and 5 were children. The onsets of lesions were respectively faces （4/15）, oral mucosa （ 3/15 ） and lower limbs （3/15）, Twelve cases were involved in trunks and extremities, blisters of 14 cases arose from the erythema or normal skin and 6 of them were annular or semicircular. Histopathologic examinations showed subepidermal/subepithelial blisters or fissures in 15 cases. Direct immunofluorescence（DIF） showed continuous linear deposition of IgAat the basement membrane zone（BMZ）. Thirteen cases were cured by corticosteroid, 2 cases had side effects of dapsone （DDS）. Conclusion The onsets of LABD mostly occur on faces, oral mucosa, lower limbs and involve in trunks and extremities in our case series. Blisters are annular form or isolated. Histopa- thology shows subepidermal/subepithelial blisters or fissures, Deposition of IgA with a linear pattern is found along the BMZ by DIF, And DDS, corticosteroid, and sulfonamides could be chosen to treat patient with LABD.
出处
《中国皮肤性病学杂志》
CAS
北大核心
2012年第10期896-899,共4页
The Chinese Journal of Dermatovenereology
