Powell J, Kirtschig G, Allen J, Dean D, Wojnarowska F
Department of Dermatology, The Churchill, Oxford Radcliffe Hospitals, Headington, Oxford OX3 7LJ, UK.
Br J Dermatol. 2001 Apr;144(4):769-74. doi: 10.1046/j.1365-2133.2001.04131.x.
Immunobullous diseases are uncommon in childhood. In contrast to adults, the most commonly seen is IgA-mediated chronic bullous disease of childhood (CBDC), while IgG-mediated bullous pemphigoid (BP), cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA) are rare. We have demonstrated both IgG and IgA autoantibodies to basement membrane zone target antigens in eight children with 'mixed immunobullous disease of childhood'.
To elucidate whether a dual antibody response makes these patients distinct regarding their presentation, immunopathology, course and prognosis.
We compared the eight children showing the double antibody response with 62 children with CBDC, BP, CP and EBA in whom only one antibody isotype was demonstrated. Clinical information at presentation, clinical course and response to treatment were recorded, and immunoblotting and direct and indirect immunofluorescence (IF) were performed.
Six of the eight patients presented with clinical features of CBDC. In two others, it was uncertain whether they had CBDC or BP. Seven of the eight demonstrated a dual antibody response on indirect IF and three on direct IF. Immunoblotting revealed a variety of epidermal and dermal target antigens (BP230, BP180, 97-kDa protein and laminin 5). Five of the eight responded well to dapsone, two to sulphonamides, and one to systemic erythromycin alone. The clinical course was not protracted. Five are in remission 1-4 years following treatment, and three still have active disease suppressed by treatment after 6 months-2 years.
Although we do not know why these children have 'mixed immunobullous disease' (the dual antibody response), our results indicate that the presence of IgA is associated with a good response to treatment with antimicrobials (dapsone, sulphonamides, erythromycin), and the clinical course is no more protracted than that found in children with a single antibody response.
免疫性大疱性疾病在儿童时期并不常见。与成人不同，儿童中最常见的是IgA介导的儿童慢性大疱性疾病（CBDC），而IgG介导的大疱性类天疱疮（BP）、瘢痕性类天疱疮（CP）和获得性大疱性表皮松解症（EBA）则较为罕见。我们在8例患有“儿童混合性免疫性大疱性疾病”的儿童中发现了针对基底膜带靶抗原的IgG和IgA自身抗体。
阐明双重抗体反应是否使这些患者在临床表现、免疫病理学、病程和预后方面具有独特性。
我们将8例显示双重抗体反应的儿童与62例仅显示一种抗体亚型的CBDC、BP、CP和EBA儿童进行了比较。记录了就诊时的临床信息、临床病程和治疗反应，并进行了免疫印迹以及直接和间接免疫荧光（IF）检测。
8例患者中有6例表现出CBDC的临床特征。另外2例患者，尚不确定他们患有CBDC还是BP。8例中有7例在间接IF上显示双重抗体反应，3例在直接IF上显示双重抗体反应。免疫印迹显示了多种表皮和真皮靶抗原（BP230、BP180、97-kDa蛋白和层粘连蛋白5）。8例中有5例对氨苯砜反应良好，2例对磺胺类药物反应良好，1例仅对全身性红霉素反应良好。临床病程并不迁延。5例在治疗后1 - 4年缓解，3例在6个月至2年后仍有经治疗控制的活动性疾病。
虽然我们尚不清楚为什么这些儿童会患有“混合性免疫性大疱性疾病”（双重抗体反应），但我们的结果表明，IgA的存在与对抗菌药物（氨苯砜、磺胺类药物、红霉素）治疗的良好反应相关，并且临床病程并不比单一抗体反应的儿童更迁延。
