Department of Dermatology, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain.
Dermatology. 2010 Aug;221(1):13-6. doi: 10.1159/000287254. Epub 2010 Apr 9.
Pemphigus vulgaris is an infrequent but life-threatening autoimmune blistering disease that is rare in the pediatric age. Although the mainstay of therapy for childhood pemphigus vulgaris (CPV) is steroids, adjuvant immunosuppressive drugs are often needed to control the disease. Thus, an important part of CPV morbidity can be related to treatment. We report the youngest CPV patient described in the English literature, an 18-month-old boy, who has been followed up for 16 years. During this period, the patient has received several immunosuppressive therapies with variable response. He has finally achieved a long-lasting and complete remission with rituximab. Successful treatment with rituximab has previously been reported in 6 cases of CPV, in whom rituximab presented a good side effect profile. Our patient has experienced a chronic and severe clinical course with multiple flares eventually developing vegetative lesions. Since he presented refractoriness to multiple therapies, we administered rituximab. The introduction of this drug led to a dramatic clinical response and a long-term clinical remission. Based on the experience of this case and the data reported in the literature, we believe that rituximab may be a safe and efficacious agent for the treatment of severe CPV.
寻常性天疱疮是一种罕见但危及生命的自身免疫性水疱病，在儿科中较为少见。虽然儿童寻常性天疱疮（CPV）的主要治疗方法是类固醇，但为了控制疾病，通常还需要辅助免疫抑制药物。因此，CPV 发病率的一个重要部分可能与治疗有关。我们报告了文献中描述的最年轻的 CPV 患者，一名 18 个月大的男孩，他已经接受了 16 年的随访。在此期间，该患者接受了多种免疫抑制治疗，但反应不一。最终，他使用利妥昔单抗实现了长期完全缓解。此前已有 6 例 CPV 患者成功接受利妥昔单抗治疗的报道，这些患者的利妥昔单抗具有良好的副作用特征。我们的患者经历了慢性和严重的临床病程，多次发作最终发展为植物性病变。由于他对多种治疗方法均无反应，我们给予了利妥昔单抗。该药物的引入带来了显著的临床反应和长期的临床缓解。基于该病例的经验和文献报告的数据，我们认为利妥昔单抗可能是治疗严重 CPV 的一种安全有效的药物。
