PDF(Pubmed)
Abstract：
Pemphigus vulgaris (PV) is a rare, potentially lethal blistering disease typically occurring in adulthood and characterized by autoantibodies directed against mucocutaneous desmosomal proteins. Clinically, flaccid vesicles, bullae and erosions after breakage are the main clinical features. According to the literature, the incidence of PV is rare in the pediatric population, ranging from 1 to 4% of reported cases. We describe an interesting case of a 12-year-old boy with severe PV that was referred to our university hospital for a mucocutaneous disease resistant to anti-infective therapy. Following the appearance of bullous lesions on the skin, antibody screening for autoimmune diseases showed positivity for PV and corticosteroid therapy was started. In view of the numerous adverse effects, we decided to set up biological therapy with rituximab, which was interrupted due to the onset of an urticarial reaction. Further second-line therapies were therefore attempted, with only a partial response. For this reason, a desensitizing therapy with rituximab was decided, thus allowing a clear improvement in the clinical picture and quality of life of the patient. To the best of our knowledge, this is the first report of a child with severe PV resistant to conventional therapies and with an urticarial reaction to rituximab. This case highlights that despite PV being extremely rare in the pediatric population, this diagnosis should not be entirely discounted. In case of severe clinical manifestations, rituximab represents a valid option in children and desensitization tests should be recommended in the presence of hypersensitivity to this drug.
摘要：
寻常型天疱疮(PV)是一种罕见的，潜在致命性水疱病通常发生在成年期，其特征是针对皮肤粘膜桥粒蛋白的自身抗体。临床上,松弛性囊泡，大疱和破裂后的糜烂是主要的临床特征。根据文献,PV的发病率在儿科人群中很少见，占报告病例的1％至4％。我们描述了一个有趣的病例，一个12岁的男孩患有严重的PV，该男孩因抗感染治疗的皮肤粘膜疾病而被转诊到我们的大学医院。皮肤上出现大疱性病变后，自身免疫性疾病的抗体筛查显示PV阳性,开始使用皮质类固醇治疗.鉴于众多不利影响，我们决定使用利妥昔单抗进行生物治疗，由于荨麻疹反应的发作而中断。因此尝试了进一步的二线疗法，只有部分回应。出于这个原因,利妥昔单抗的脱敏疗法被决定，从而使患者的临床表现和生活质量得到明显改善。据我们所知,这是首次报道一例严重PV对常规治疗耐药,对利妥昔单抗有荨麻疹反应的儿童.这个病例突出表明，尽管PV在儿科人群中极为罕见，这种诊断不应完全低估。如果有严重的临床表现，利妥昔单抗是儿童的有效选择，在对该药物过敏的情况下，应建议进行脱敏试验。
