Necrobiotic xanthogranuloma
(Redirected from Necrobiotic xanthogranuloma with paraproteinemia)
Alternate names[edit | edit source]
NXG
Definition[edit | edit source]
Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults. Xanthogranulomas are lesions made of immune cells known as a histiocytes. The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope.
Signs and symptoms[edit | edit source]
- 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.
- As these blood disorders may arise years after the first NXG lesions appear, lifelong follow-up may be recommended.
- 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.
- 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.
Treatment[edit | edit source]
- 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.
See also[edit | edit source]
External links[edit | edit source]
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