波特浮肿瘤 (PPT) 是一种涉及额骨的罕见感染，由 Percival Pott 爵士于 250 多年前首次描述。它可以表现为颅内扩展和严重的神经系统疾病。色素失禁 (IP) 是一种罕见的遗传性皮肤病，在男性中在产前是致命的，在女性中临床表现出来。 IP 与反复感染和免疫功能障碍/抑制有关。我们报告了一例 IP 儿童出现波特氏浮肿肿瘤的病例。我们还对与免疫功能障碍相关的 PPT 病例进行了文献综述。我们讨论了这些病变的临床表现、诊断和治疗。我们确定了 12 例与免疫功能障碍/抑制相关的 PPT 病例。糖尿病是最常见的原因，其次是医源性免疫抑制。手术是治疗 PPT 的标准治疗方法，并且讨论了有或没有颅内受累的 PPT 的治疗，特别是在潜在的免疫功能障碍/抑制的情况下。PPT 仍然是一种罕见但并非罕见的诊断，通常需要神经外科干预。免疫功能障碍/抑制是可能诱发 PPT 的另一个危险因素。应采取早期积极的管理以获得最佳结果。© 2024。作者获得 Springer-Verlag GmbH 德国（Springer Nature 旗下公司）的独家许可。

Pott's puffy tumor (PPT) is an uncommon infection involving the frontal bone, first described by Sir Percival Pott more than 250 years ago. It can present with intracranial extension and serious neurological morbidity. Incontinentia pigmenti (IP) is a rare inherited genodermatosis that is lethal prenatally in males and manifests clinically in females. IP is associated with recurrent infections and immune dysfunction/suppression.We report a case of Pott's puffy tumor presenting in a child with IP. We also performed a literature review of reported cases of PPT associated with immune dysfunction. We discuss the clinical presentation, diagnosis, and management of these lesions.We identified 12 cases of PPT associated with immune dysfunction/suppression. Diabetes was the most commonly identified cause followed by iatrogenic immunosuppression. Surgery is the standard treatment for managing PPT and the management of PPT with and without intracranial involvement, particularly in the context of underlying immune dysfunction/suppression, is discussed.PPT remains a rare but not infrequent diagnosis, often requiring neurosurgical intervention. Immune dysfunction/suppression is an additional risk factor that may predispose to PPT. Early and aggressive management should be instituted for optimal outcome.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.