Colombo M, Volpini S, Orini S, Fazi M C, Vettore E, Tedoldi S, Cappellaro E
Unità Operativa di Pediatria, Presidio Ospedaliero di Manerbio, Manerbio Brescia, Italy.
Minerva Pediatr. 2008 Jun;60(3):351-3.
Linear IgA bullous dermatosis is an acquired subepidermal blistering disease which belongs to bullous autoimmune diseases, along with dermatitis herpetiformis and bullous pemphigoid. Inflammatory blisters are the main clinical characteristics and the areas of common involvement are: perioral region, abdomen, perineum, buttocks and the interior side of thighs. Essential for the diagnosis is to find by direct immunofluorescence the presence of a linear band of IgA antibodies at the level of the basement membrane. We present the case of a 5 year-old Moroccan girl which arrived at our First Aid Department for bullous dermatitis, localized mainly on the abdomen, legs and thighs. During a short stay in Morocco, a month before, the little girl was stung by an insect and developed bullous dermatitis by a residual lesion. The child was in a good state of health but blood exams showed an increase of total IgE antibodies. The girl was admitted and during her hospitalisation we made a skin biopsy which led to a diagnosis of linear IgA dermatosis. She began a steroid therapy and there was a progressive regression of the lesions. At present, she does not take medicines anymore, she feels well and is submitted to ambulatory medical follow-up.
线状IgA大疱性皮肤病是一种获得性表皮下大疱性疾病，与疱疹样皮炎和大疱性类天疱疮同属大疱性自身免疫性疾病。炎性水疱是其主要临床特征，常见受累部位包括：口周区域、腹部、会阴、臀部及大腿内侧。诊断的关键是通过直接免疫荧光法在基底膜水平发现IgA抗体的线状条带。我们报告一例5岁摩洛哥女孩的病例，她因大疱性皮炎来到我们的急救科，主要病变位于腹部、腿部和大腿。一个月前在摩洛哥短暂停留期间，小女孩被昆虫叮咬，因残留损伤而出现大疱性皮炎。患儿健康状况良好，但血液检查显示总IgE抗体升高。女孩入院后，住院期间我们进行了皮肤活检，诊断为线状IgA皮肤病。她开始接受类固醇治疗，皮损逐渐消退。目前，她不再服药，感觉良好，正在接受门诊医学随访。
