Rare Hematology News

Disease Profile

Necrobiotic xanthogranuloma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

D76.3

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

NXG

Categories

Skin Diseases

Summary

Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults.[1][2][3] Xanthogranulomas are lesions made of immune cells known as a histiocytes.[4] The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope.[1][2] The typical lesion is a yellow, thickened, or raised lesion (plaque) located around the eyes. In most cases the lesions are associated with conditions in which abnormal proteins are found in the blood (monoclonal gammopathies). NXG is also sometimes associated with blood cancers or lymphoproliferative disorders. [1][2][3] As these blood disorders may arise years after the first NXG lesions appear, lifelong follow-up may be recommended.[2] Approximately 50% of people with NXG have problems with their eyes, such as burning or itching, blurred vision, double vision, bulging of the eyeball, drooping of the eyelid, and restricted eye movement. [2] In most cases, other parts of the body, such as the trunk, legs, face, and arms, are also involved. Internal organs may sometimes be affected.[3] Treatment of NXG can be challenging, but is usually necessary to minimize the risk of skin ulcers and scarring. First-line therapy may include drugs used to treat cancer (such as chlorambucil and melphalan). Additional treatment options include corticosteroids, intravenous immunoglobulin, lenalidomide, interferon, radiation therapy, and surgery. In some cases, the symptoms of NXG may return after treatment. If NXG is associated with a blood cancer or a lymphoproliferative disorder, treatment focuses on the blood disorder.[2][3]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Necrobiotic xanthogranuloma. Click on the link to view a sample search on this topic.

References

  1. Cheng, Harriet. Necrobiotic xanthogranuloma pathology. DermNet New Zealand. 2013; https://www.dermnetnz.org/topics/necrobiotic-xanthogranuloma-pathology.
  2. Girisha BS, Holla AP, Fernandes M, Noronha TM. Necrobiotic Xanthogranuloma. Journal of Cutaneous and Aesthetic Surgery. 2012; 5(1):43-45. .https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3339130.
  3. Schadt C. and Jacobsen E. Necrobiotic xanthogranuloma. UpToDate. May, 2016; https://www.uptodate.com/contents/necrobiotic-xanthogranuloma.
  4. Achar A, Naskar B, Mondal PC, Pal M. Multiple Generalized Xanthogranuloma In Adult: Case Report And Treatment. Indian Journal of Dermatology. 2011; 56(2):197-99. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3108521.