摘要
目的了解儿童线状IgA大疱性皮病的临床特点,提高临床医生对本病的认识,以减少误诊和漏诊。方法对9例儿童线状IgA大疱性皮病的临床资料、组织病理、免疫病理、误诊和治疗进行回顾性分析。结果 9例患儿中,男4例,女5例。皮损临床表现:红斑基础上或外观正常的皮肤上出现水疱和大疱,疱壁紧张,疱液清亮,尼氏征(-)。皮损组织病理均见表皮下疱,直接免疫荧光见IgA线状沉积于基底膜带。误诊为多形红斑1例、湿疹1例、丘疹性荨麻疹1例、大疱性类天疱疮(BP)2例、疱疹样皮炎(DH)2例和获得性大疱性表皮松解症(EBA)2例。结论儿童线状IgA大疱性皮病临床少见,易被误诊或漏诊,直接免疫荧光见IgA线状沉积于基底膜带对诊断本病有重要意义。
Objective To analyze the clinical features of linear IgA bullous dermatosis （LABD） of childhood, and im-prove the understanding of clinician so as to reduce misdiagnosis and missed diagnosis. Methods Clinical data, histopathological characteristics, immunofluorescence, misdiagnosis and treatment of 9 cases with linear IgA bullous dermatosis of childhood were retrospectively analized. Results Out of the 9 cases,4 were male and 5 were female. Clinical manifestations of skin lesions were blisters which appeared on the erythema or normal skin. The blisters were tense and the blister fluid was clear. Nikosky~ sign was negative. Histopatho- logical examination results of 9 cases revealed subepidermal blister. Direct immunofluorescence examinations showed linear depositions of IgA along the basement membrane zone. Nine cases were misdiagnosed as one case of erythema multifonne, one case of eczema, one case of papular urticaria, two cases of bullous pemphig- oid（BP）, two cases of dermatitis herpetifornis （DH） and two cases of epidermolysis bullosa acquisita （EBA）. Conclusion Linear IgA bullous dermatosis of childhood is rare clinically. It is easily misdiagnosed and missed diagnosed. It is important for the diagnosis of LABD that linear depositions of IgA along the base- ment membrane zone is found on direct immunofluorescence.
出处
《中国皮肤性病学杂志》
CAS
北大核心
2013年第5期478-479,481,共3页
The Chinese Journal of Dermatovenereology
关键词
线状IGA大疱性皮病
儿童
误诊
治疗
Linear IgA bullous dermatosis （LABD）
Childhood
Misdiagnose
Treatment
