Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany.
Department of Nuclear Medicine, Technical University Munich, Ismaninger Strasse 22, 81675, Munich, Germany.
Orphanet J Rare Dis. 2022 Mar 24;17(1):132. doi: 10.1186/s13023-022-02291-z.
Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature.
To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies.
All reported papers in the literature were screened for systemic treatments of NXG. Papers without proper description of the therapies, papers describing topical therapy, and articles without assessment of effectiveness were excluded. Subsequently, we analyzed 79 papers and a total of 175 cases.
The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids.
Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG.
尽管已经提出了许多系统性疗法来治疗坏死性黄色肉芽肿（NXG），但文献中尚无对此主题的系统综述。
综述所有关于 NXG 系统性治疗的现有文献，以确定最有效的治疗方法。
对文献中所有报道的 NXG 系统性治疗进行筛选。排除未详细描述治疗方法、描述局部治疗方法和未评估疗效的论文。随后，我们分析了 79 篇论文和总共 175 例病例。
NXG 最有效的治疗方法是静脉注射免疫球蛋白（IVIG）、皮质类固醇和包括皮质类固醇在内的联合疗法。
因此，皮质类固醇和 IVIG 应被视为 NXG 患者的一线治疗方法。
