Reyes-Baraona Francisco, Andino Romina, Carrasco Juan Eduardo, Arriagada Camila, Guerrero Silvia
Consultorio Pudahuel.
Servicio de Dermatología, Pontificia Universidad Católica, Chile.
Arch Argent Pediatr. 2014 Apr;112(2):e57-60. doi: 10.5546/aap.2014.e57.
Linear IgA bullous dermatosis is a rare acquired autoinmune vesiculobullous disease characterized by linear IgA deposit on the dermo-epidermal basement membrane observed with direct inmunofluorescence. The characteristic lesions are vesicles and tense serous bullae, which most often are grouped giving a "cluster of jewels" appearance. Differential diagnosis must be established with other autoimmune dermatosis, such as dermatitis herpetiformis and bullous pemphigoid. Dapsone is the first line therapy, with excellent response in a short period. This is a benign disease that tends to wax and wane in severity until it disappears spontaneously. We report the case of a 5-year-old girl presenting with bullous lesions, being diagnosed a linear IgA bullous dermatosis, with excellent response to dapsone in less than 2 weeks.
线状IgA大疱性皮肤病是一种罕见的获得性自身免疫性水疱大疱性疾病，其特征是在直接免疫荧光检查中观察到真皮表皮基底膜上有线状IgA沉积。特征性损害为水疱和紧张性浆液性大疱，最常见的是成群出现，呈“宝石簇”外观。必须与其他自身免疫性皮肤病进行鉴别诊断，如疱疹样皮炎和大疱性类天疱疮。氨苯砜是一线治疗药物，短期内反应良好。这是一种良性疾病，病情严重程度往往有波动，直至自行消失。我们报告一例5岁女童出现水疱性损害，被诊断为线状IgA大疱性皮肤病，在不到2周的时间内对氨苯砜反应良好。
