Citation, DOI, disclosures and article data
Citation:
Deng F, Rasuli B, Knipe H, et al. Necrobiotic xanthogranuloma. Reference article, Radiopaedia.org (Accessed on 20 Feb 2025) https://doi.org/10.53347/rID-63519
Necrobiotic xanthogranulomas are a rare form of cutaneous non-Langerhans cell histiocytosis.
The condition occurs mostly in older adults 2.
Patients demonstrate cutaneous papules that develop into large, firm plaques, papules and nodules, which are often yellow 1. The most common site is the periorbital skin 1. Involvement of the periorbital soft tissues can cause ptosis, exophthalmos, diplopia, restricted ocular movement, and pain 3.
The exact etiology of the condition is unknown due to its rarity 2.
Associations
Most patients have a monoclonal gammopathy, which has the potential to develop into multiple myeloma or other lymphoproliferative disorders 1.
Cross-sectional imaging of the face on CT or MRI demonstrates soft tissue thickening or masses in the periorbital region. Internal organs (e.g. the liver) may also be affected.
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1. Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel-Wahab O, Allen CE, Charlotte F, Diamond EL, Egeler RM, Fischer A, Herrera JG, Henter JI, Janku F, Merad M, Picarsic J, Rodriguez-Galindo C, Rollins BJ, Tazi A, Vassallo R, Weiss LM. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. (2016) Blood. 127 (22): 2672-81. doi:10.1182/blood-2016-01-690636 - Pubmed
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2. Girisha BS, Holla AP, Fernandes M, Noronha TM. Necrobiotic xanthogranuloma. (2012) Journal of Cutaneous and Aesthetic Surgery. 5 (1): 43. doi:10.4103/0974-2077.94341 - Pubmed
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3. Achar A, Naskar B, Mondal PC, Pal M. Multiple generalized xanthogranuloma in adult: case report and treatment. (2011) Indian journal of dermatology. 56 (2): 197-9. doi:10.4103/0019-5154.80416 - Pubmed
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