Department of Dermatology and Reference Centre for Genodermatoses and Rare Skin Diseases (MAGEC), Hôpital Universitaire Necker-Enfants Malades, Assistance publique-Hôpitaux de Paris, Université Paris Descartes - Sorbonne Paris Cité, Paris, France.
Department of Pathology, Hôpital Universitaire Necker-Enfants Malades, Assistance publique-Hôpitaux de Paris, Université Paris Descartes -Sorbonne Paris Cité, Paris, France.
J Eur Acad Dermatol Venereol. 2019 Jun;33(6):1158-1163. doi: 10.1111/jdv.15456. Epub 2019 Mar 14.
Autoimmune bullous dermatoses (AIBDs) in children are uncommon, and their long-term evolution remains unknown.
The aim of this retrospective study was to characterize the long-term prognosis of AIBDs that started during childhood.
We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBDs. The long-term outcome was obtained through a phone call questionnaire.
Sixty-three patients were included from January 1993 to December 2015, 34 female and 29 males: 27 Linear immunoglobulin A disease (LAD), 12 bullous pemphigoid (BP), 12 pemphigus, 8 herpetiform dermatitis (DH) and 4 epidermolysis bullosa aquisita (EBA). The mean age was 4.7 years old. Twenty-five patients were lost during the follow-up. For the 38 remaining patients, the mean follow-up duration for all pathologies was 6.6 years. Twenty-nine of them had at least one relapse. Late relapses were observed in two cases of DH and six cases of pemphigus (7-34 months). The mean treatment duration was 30.6 months with variability according to the AIBDs. Topical corticosteroids were used alone, effectively, for seven patients and in association with other treatment in 19 patients in complete remission. Complete remission was noted in 34/38 children with a follow-up of 4.4 years (0.08-19.5). The mean duration to complete remission was 30.5 months (6-114 months). Late nasal synechiae were reported in one EBA only. There was no significant associated comorbidity, but an association with a primary immune deficiency (PID) was observed in two cases.
Childhood AIBDs appear to be of good overall prognosis but a long-term follow-up is mandatory, as relapses can be late, except for BP. The use of topical corticosteroids is frequently effective alone or in association. The association with PID leads to think about the possibility of a possible underlying dysimmunity in the child.
儿童自身免疫性大疱性皮肤病（AIBD）并不常见，其长期演变尚不清楚。
本回顾性研究旨在描述儿童时期发病的 AIBD 的长期预后。
我们进行了一项单中心回顾性研究，纳入了所有患有 AIBD 的儿童患者。通过电话问卷调查获得长期结果。
1993 年 1 月至 2015 年 12 月，我们共纳入 63 例患者，其中女 34 例，男 29 例：27 例线性免疫球蛋白 A 疾病（LAD），12 例大疱性类天疱疮（BP），12 例天疱疮，8 例疱疹样皮炎（DH）和 4 例获得性大疱性表皮松解症（EBA）。平均年龄为 4.7 岁。25 例患者在随访过程中失访。在其余 38 例患者中，所有患者的平均随访时间为 6.6 年。其中 29 例至少有一次复发。2 例 DH 和 6 例天疱疮（7-34 个月）出现迟发性复发。平均治疗时间为 30.6 个月，不同 AIBD 患者间存在差异。7 例患者单独使用外用皮质类固醇有效，19 例患者完全缓解时联合其他治疗。38 例中有 34 例（4.4 年随访时 0.08-19.5 年）完全缓解。完全缓解的平均时间为 30.5 个月（6-114 个月）。仅 1 例 EBA 患者出现晚期鼻粘连。无明显相关合并症，但有 2 例与原发性免疫缺陷（PID）相关。
儿童 AIBD 总体预后良好，但需要长期随访，因为除 BP 外，迟发性复发仍有可能发生。单独或联合使用外用皮质类固醇通常有效。与 PID 的关联提示患儿可能存在潜在的免疫失调。
