PDF(Pubmed)
Abstract：
Epidermolysis bullosa acquisita (EBA) rarely develops in childhood. This study retrospectively recruited paediatric patients with EBA (age ≤ 16 years), diagnosed by clinical and histopathological features and results of immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA), and reviews their clinical manifestations, histopathology, immunological features, and responses to various treatments. All 7 included patients presented with inflammatory EBA. Among them, 3 had a bullous pemphigoid-like phenotype. Pathologically, in addition to dermal-epidermal blistering, in all patients, the distribution of neutrophils was superficial perivascular or interstitial, or in the dermal papilla. Mixed neutrophils and eosinophils were detected in 2 of the 3 patients with bullous pemphigoid-like phenotypes. In addition to treatment with glucocorticoids, dapsone was administered in 4 patients, while thalidomide and sulfasalazine were administered in 1 patient. All patients responded to the these therapies. Relapse was mainly due to reduction and cessation of glucocorticoids. In conclusion, EBA in childhood may be unique, and thus distinct from its adult counterpart. Specific treatment and follow-up protocols are required for therapy of this rare autoimmune skin disease in children.
摘要：
儿童时期很少发生大疱性表皮松解症（EBA）。这项研究回顾性招募了EBA儿科患者（年龄≤16岁），通过临床和组织病理学特征以及免疫荧光结果诊断，免疫印迹和酶联免疫吸附测定(ELISA),回顾他们的临床表现，组织病理学,免疫学特征，以及对各种治疗的反应。所有7名患者均患有炎性EBA。其中,3具有大疱性类天疱疮样表型。病理上,除了真皮表皮起泡，在所有患者中，中性粒细胞分布在浅表血管周围或间质，或真皮乳头。在3例大疱性类天疱疮表型患者中，有2例检测到混合的中性粒细胞和嗜酸性粒细胞。除了糖皮质激素治疗，4例患者服用氨苯砜，1例患者给予沙利度胺和柳氮磺胺吡啶。所有患者都对这些疗法有反应。复发主要是由于糖皮质激素的减少和停止。总之,童年的EBA可能是独一无二的，因此不同于它的成人对应物。治疗这种罕见的儿童自身免疫性皮肤病需要特定的治疗和随访方案。
