在文献中，有一些报道描述了副肿瘤性天疱疮（PNP）和卡斯特尔曼病（CD）之间的关联，但没有提出一致的诊断治疗方法。本研究旨在报告一例病例，并探讨儿科患者中PNP和CD之间的关系，重点关注临床表现、组织病理学发现、治疗和结果，以找到早期诊断的依据。我们报告一名13岁的女孩的临床病例，她患有PNP和CD，经过利妥昔单抗和西替单抗治疗以控制症状。回顾文献，儿童年龄段已经描述了20个临床病例。诊断可能具有挑战性，需要平均3个月的时间（范围为3周至2年）。在所有病例中，最初的表现是黏膜皮肤病变，特别是口腔病变，对传统治疗反应差。全身症状也可能存在。治疗方法仍在讨论中，没有一致的意见。几乎所有患者都接受了皮质类固醇治疗，但治疗效果不佳。其他药物，包括硫唑嘌呤、甲氨蝶呤、环孢素和单克隆抗体已用于疾病控制的评估。进一步的研究和实验性试验迫切需要确定诊断标准和治疗方案。©2023年作者（们）。

In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis.We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms.Reviewing literature, 20 clinical cases have been described in the pediatric age. Diagnosis may be challenging, requiring an average of 3 months (range from 3 weeks to 2 years). In all cases, the initial manifestations were mucocutaneous lesions, especially oral lesions with poor response to conventional treatment. Systemic symptoms may be present as well. Therapeutical approach is still discussed with no consensus. Almost all patients received corticosteroids with poor response. Other drugs including azathioprine, methotrexate, cyclosporine and monoclonal antibodies have been evaluated for the control of the disease. Further studies and experimental trials urge to define the diagnostic criteria and therapy protocol.© 2023. The Author(s).