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Necrobiotic xanthogranuloma: a 30-year single-center experience
Talal Hilal1, David J DiCaudo2,3, Suzanne M Connolly2
1Division of Hematology/Oncology, Mayo Clinic, 5777 E. Mayo Boulevard, Phoenix, AZ, 85054, USA. hilal.talal@mayo.edu.
Necrobiotic xanthogranuloma (NXG) is a rare histiocytosis often linked to monoclonal proteins and affecting multiple organs, particularly the periorbital area. Most patients respond well to treatments like chlorambucil, often with corticosteroids.
Area of Science:
- Dermatology
- Hematology
- Oncology
Background:
- Necrobiotic xanthogranuloma (NXG) is a rare, non-Langerhans cell histiocytosis.
- It is characterized by xanthomatous lesions and often associated with systemic conditions.
Purpose of the Study:
- To characterize the clinical features of NXG.
- To identify associated disorders and evaluate treatment outcomes.
Main Methods:
- Retrospective review of pathologically confirmed NXG cases.
- Data extraction on clinical, laboratory, and treatment information.
Main Results:
- Nineteen patients were identified with a mean age of 54 years.
- 84% had detectable monoclonal protein; 53% had periorbital lesions.
- Extracutaneous manifestations (liver, sinuses) and hematologic malignancies (26%) were common.
Conclusions:
- NXG is a reactive histiocytic disorder involving multiple organ systems.
- High clinical suspicion is crucial for diagnosis.
- Treatment should consider comorbidities and disease patterns.
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