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Systemic therapy of necrobiotic xanthogranuloma: a systematic review

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. Department of Radiology, Technical University Munich, Ismaninger Strasse 22, 81675, Munich, Germany. Department of Otorhinolaryngology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany. Department of Ophthalmology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany. Department of Internal Medicine III, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany. Department of Otorhinolaryngology, Ludwig Maximilians University Munich, Marchioninistr. 15, 81377, Munich, Germany. Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany. Stephan.schreml@ukr.de.

Orphanet journal of rare diseases. 2022;17(1):132
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Abstract
BACKGROUND Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature. OBJECTIVE To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies. METHODS 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. RESULTS The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids. CONCLUSIONS Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG.
Study details
Study Design : Systematic Review
Language : eng
Credits : Bibliographic data from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine