University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, New York, USA.
Neurosurgery, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, New York, USA.
BMJ Case Rep. 2023 Aug 30;16(8):e254122. doi: 10.1136/bcr-2022-254122.
Necrobiotic xanthogranuloma is a condition that was first identified in 1980 based on its characteristic histological identity, and it has been known since then as a dermatologic manifestation of an underlying systemic dysproteinemia. Intracranial manifestation is a rare presentation of this condition and has been reported only once in its more than 40 years of existence. Herein and to our knowledge, we report the second observation of an intracranial manifestation and, surprisingly, the first case without the expected dermatologic and systemic dysproteinemia associations. This case identifies an existing knowledge gap in our understanding of necrobiotic xanthogranuloma and emphasises the need for further research into understanding the presentation, comorbidities and management of this condition.
坏死性黄色肉芽肿是一种病症,于 1980 年根据其特征性组织学特征首次确定,此后一直被认为是潜在系统性副蛋白血症的皮肤表现。颅内表现是这种病症罕见的表现形式,在其 40 多年的存在过程中仅报告过一次。在此,据我们所知,我们报告了第二个颅内表现的观察结果,令人惊讶的是,这是第一个没有预期的皮肤和系统性副蛋白血症相关的病例。该病例确定了我们对坏死性黄色肉芽肿的理解存在一个知识空白,并强调需要进一步研究以了解这种病症的表现、合并症和管理。