Monia Kharfi, Aida Khaled, Amel Karaa, Ines Zaraa, Becima Fazaa, Ridha Kamoun Mohamed
Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.
Indian J Dermatol. 2011 Mar;56(2):153-9. doi: 10.4103/0019-5154.80406.
Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa.
We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children.
We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population.
Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients.
This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.
儿童线状IgA大疱性皮肤病（LAD）在非洲相对常见。
我们开展这项研究以评估该病在突尼斯儿童自身免疫性大疱性疾病（AIBDs）中的发病频率。
我们进行了一项为期32年的回顾性研究（1976年1月至2007年12月）。纳入在突尼斯查尔斯·尼科勒医院就诊的患有慢性获得性大疱性疾病且皮损周围皮肤直接免疫荧光（DIF）显示线状IgA免疫球蛋白沉积的儿童作为研究对象。
选取了31例LAD患儿，占同期所选全部儿童AIBDs的65.9%，平均年龄5.5岁，男女比例（男/女）为2.4。大多数患儿有泛发性皮疹（28/31），面部、盆腔区域、臀部和四肢更为明显。仅4例患儿（12.9%）出现黏膜损害。疾病平均病程为14个月。DIF显示所有患者真皮 - 表皮交界处有线状IgA沉积。还可见IgG、IgM和补体（20/31）。间接免疫荧光在67%的病例中为阴性。8例患者对氨苯砜有反应；然而，7例患儿必须加用泼尼松来控制病情，另外4例加用红霉素。76.1%的患者实现了长期缓解。
本研究证实LAD是突尼斯儿童中最常见的AIBD，常见于学龄前男性。无论使用何种药物，经常可观察到长期缓解。
