儿童获得性大疱表皮松解症(附4例报道)

Epidermolysis bullosa acquisita in childhood: report of 4 cases

儿童获得性大疱表皮松解症 (EBA)是一种自身免疫性表皮下大疱病，比较少见。本文报道 4例儿童 EBA，年龄为 1、 4、 7、 14岁，均为男性。临床上似营养不良性大疱表皮松解症 2例，儿童型线形 IgA大疱病 2例。 3例有口腔损害，伴血疱，萎缩性瘢痕和粟丘疹。病理为表皮下水疱。 DIF均示 IgG和 C3呈线状分布于基底膜带， 3例还见 IgA， 1例见 IgM沉积于基底膜带。盐裂 DIF示 3例 IgG沉积于真皮侧。 IIF示 3例 IgG和 1例 IgA抗基底膜抗体，免疫印迹测得 4例均结合 290kD蛋白条带。应用皮质类固醇， DDS或中药雷公藤能很好控制病情。

Epidermolysis bullosa acquisita(EBA) is an autoimmune subepidermal bullous disease. This disease is rare in childhood. We report 4 new cases of EBA in children, 1,4,7and 14 years old respectively, all 4 children were male, with a clinical phenotype resembling dystrophic epidermolysis bullosa in 2 cases and linear IgA bullous disease of children in 2 cases. Mucosal involvement was present in 3 cases with bloody blisters, atrophic scar and milia. The results of histologic examination showed subepidermal blister in all cases. Direct immunofluorescence(DIF) showed linear band of IgG and C3 at the dermoepidermal junction in all cases, with IgA deposits in 3 cases and IgM deposits in one case. The results of DIF on saline- split skin were positive with IgG deposits on the dermal side of the cleavage. Indirect immunofluorescence (IIF) showed circulation anti- basement membrane autoantibodies of the IgG type in 3 of 4 cases, and the IgA type in 1 of 4 cases. Western blot analysis on dermal extract showed the circulation autoantibodies recognized the band of 290 kD in all 4 cases. The combination of prdenisone and dapsone or Tripterygium willrordii (hineses herb) appears to be effective.