(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.
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 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
). Additional treatment options include corticosteroids
, intravenous immunoglobulin
, 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.
Last updated: 11/13/2017