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Orphanet Journal of Rare Diseases

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Open Access 01-12-2022 | Langerhans Cell Histiocytosis | Review

Systemic therapy of necrobiotic xanthogranuloma: a systematic review

Authors: Lisa Steinhelfer, Thomas Kühnel, Herbert Jägle, Stephanie Mayer, Sigrid Karrer, Frank Haubner, Stephan Schreml

Published in: Orphanet Journal of Rare Diseases | Issue 1/2022

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.

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Title: Systemic therapy of necrobiotic xanthogranuloma: a systematic review
Authors: Lisa Steinhelfer
Thomas Kühnel
Herbert Jägle
Stephanie Mayer
Sigrid Karrer
Frank Haubner
Stephan Schreml
Publication date: 01-12-2022
Publisher: BioMed Central
Keywords: Langerhans Cell Histiocytosis
Xanthogranuloma
Systemic Therapy
Published in: Orphanet Journal of Rare Diseases / Issue 1/2022
Electronic ISSN: 1750-1172
DOI: https://doi.org/10.1186/s13023-022-02291-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Systemic therapy of necrobiotic xanthogranuloma: a systematic review

Abstract

Background

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Objective

Methods

Results

Conclusions

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