Verma Nitin, Pickles Charles, Khan Muhammad Amjad
Department of Paediatrics, Darlington Memorial Hospital, County Durham and Darlington NHS Foundation Trust, Darlington, UK.
Department of Paediatric Dermatology, Darlington Memorial Hospital, County Durham and Darlington NHS Foundation Trust, Darlington, UK.
Case Rep Pediatr. 2016;2016:5328603. doi: 10.1155/2016/5328603. Epub 2016 Dec 13.
Linear IgA disease of childhood (LAD) also known as chronic bullous disease of childhood is an autoimmune disease with IgA deposition at the basement membrane zone leading to a vesiculobullous rash. It has a clinical appearance which frequently is described as resembling "strings of pearls" or rosette-like. Diagnosis is usually clinical but sometimes biopsy is required. Dapsone is widely considered to be the first line therapy in the treatment of LAD. A 5-year-old girl presented with 4-day history of a widespread painful rash and pyrexia. The rash transformed into painful blisters. A recent contact with chickenpox was present. She remained apyrexial but hemodynamically stable and was treated as chickenpox patient with secondary infection. Due to persistent symptoms after repeated attendance she was reviewed by Dermatology team and diagnosed with linear IgA disease also known as chronic bullous disease of childhood. This was based on the presence of blistering rash with rosette appearance and string of pearl lesions. The clinical features of LAD can be difficult to distinguish from more common skin infections. Benefiting from the experience of other multidisciplinary teams can sometimes be a game changer and can lead to the correct diagnosis and treatment.
儿童线状IgA疾病（LAD），也称为儿童慢性大疱性疾病，是一种自身免疫性疾病，IgA沉积于基底膜带，导致水疱大疱性皮疹。其临床表现常被描述为类似“珍珠串”或玫瑰花结样。诊断通常基于临床症状，但有时需要进行活检。氨苯砜被广泛认为是治疗LAD的一线疗法。一名5岁女孩出现广泛疼痛性皮疹和发热4天的病史。皮疹转变为疼痛性水疱。近期有接触水痘史。她不再发热，但血流动力学稳定，被当作水痘继发感染患者进行治疗。由于多次就诊后症状持续存在，皮肤科团队对她进行了复查，诊断为线状IgA疾病，也称为儿童慢性大疱性疾病。这是基于出现具有玫瑰花结外观和珍珠串样损害的水疱性皮疹。LAD的临床特征可能难以与更常见的皮肤感染相区分。借鉴其他多学科团队的经验有时可能会带来改变，从而实现正确的诊断和治疗。
