Systemic therapy of necrobiotic xanthogranuloma: a systematic review
Lisa Steinhelfer1,2,3, Thomas Kühnel4, Herbert Jägle5
1Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany.
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.
Insights
Necrobiosis lipoidica (NXG) treatment lacks systematic review. Intravenous immunoglobulins (IVIG) and corticosteroids are most effective systemic therapies for NXG.
Area of Science:
- Dermatology
- Internal Medicine
- Systemic Therapy
Background:
- Necrobiosis lipoidica (NXG) is a rare granulomatous disease.
- Numerous systemic therapies exist, but evidence is fragmented.
- No prior systematic review has synthesized NXG systemic treatment data.
Purpose of the Study:
- To systematically review and identify effective systemic therapies for NXG.
- To provide evidence-based recommendations for NXG management.
Main Methods:
- Comprehensive literature search for systemic NXG treatments.
- Inclusion of 79 papers detailing 175 NXG cases.
- Exclusion of topical therapies and studies lacking efficacy assessment.
Main Results:
- Intravenous immunoglobulins (IVIG) demonstrated high efficacy.
- Corticosteroids, both alone and in combination, were highly effective.
- Combination therapies including corticosteroids showed significant benefits.
Conclusions:
- Corticosteroids and IVIG are recommended as first-line treatments for NXG.
- This review provides a foundation for evidence-based NXG treatment guidelines.
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