儿童型大疱性类天疱疮临床特征及诊疗特点

Clinical Characteristics and Management of Childhood Bullous Pemphigoid

目的了解儿童型大疱性类天疱疮(CBP)的临床特征及诊疗特点。方法回顾性分析郑州大学第一附属医院经治2例及既往文献病例中临床资料基本完整者17例共19例CBP的临床资料、实验室检查结果及诊疗方案等。结果 19例CBP中,男15例,女4例;年龄6个月~13岁;病程8d^4年。皮损多位于胸、腹、背部及四肢,口腔、腋窝、会阴区等黏膜及皱褶部位亦有受累,临床表现为正常或红斑基础上大小不等的张力性厚壁水疱,尼氏征阴性,多伴程度不等的瘙痒。典型组织病理可见表皮下水疱或裂隙形成,直接免疫荧光多呈基底膜带IgG和(或)C3呈线状沉积,部分病例可见IgM和IgA沉积。平均予泼尼松1.253mg/(kg·d)治疗后均见好转。随访至今未见复发。结论 CBP诊断需综合考虑临床病史、组织病理学、直接免疫荧光及盐裂实验+间接免疫荧光等检查结果,治疗首选糖皮质激素,且预后良好。

Objective To learn clinical characteristics and management of childhood bullous pemphigoid（CBP）.Methods The clinical, laboratory and treatment characteristics of 19 cases of CBP, including 2 cases from the First Affiliated Hospital of Zhengzhou University and 17 cases from domestic literatures reported before, were included included and analyzed.Results Male 15, female 4, age range of onset varies between 6 months to 13 years, while history ranged from 8 days to 4 years. Chest, abdomen, back and limbs were always involved, while oral mucosa, axillary, perineal could be involved occasionally. Tension blisters with different size, thick wall can be found on the basis of normal skin or red macule, accompanied with different degree of itch and Nikolsky sign（-）. Typical pathology shows the formation of subepidermal bullous and blister. Direct immunofluorescence demonstrates the linear deposition of IgG and（or）C3 in the basement membrane zone, while IgM、IgA also can be find sometimes. Remission has been found in all cases after the administration of predisone（average dosage 1.253mg/kg·d）, and no recurrence.Conclusion Diagnosis of CBP should be made under the comprehensive consideration of clinical history, histopathology,direct immunofluorescence and salt split technique. Glucocorticoid is the first choice to treat CBP with good prognosis.