LAUSR

Necrobiotic xanthogranuloma (NXG) disease is a rare nonLangerhans cell tissue proliferative disease that was first reported in 1980 (1). It is often associated with the abnormal proliferation of monoclonal gammopathy (M) protein. Among all cases with NXG, 50% of are immunoglobulin G (IgG)-κ type, the rarer IgG-λ type accounts for about 21%, and the remaining 12% consists of IgA, IgG, polyclonal, and unspecified types, with 17% being without paraproteinemia (2). Studies have shown that paraproteins play an essential role in granulation tissue formation (3). The disease often occurs in women over 60 years old, and the female-to-male ratio is about 1.7:2.7 (2,4). Nelson et al. (2) proposed the diagnostic criteria of NXG based on primary and secondary clinical and histopathological findings. The primary conditions include (I) yellow or orange papulonodular plaques on the skin that are (II) consistent with histopathological features demonstrating palisading granulomas with lymphoplasmacytic infiltrate and zones of necrobiosis. The secondary conditions include (I) paraproteinemia, plasmacytoid disease, and/or other related lymphoproliferative disorders; and (II) distribution in the periorbital skin. After exclusion of a foreign body, infection, or other determinable diseases, the diagnosis is established by meeting the primary conditions and at least 1 secondary condition. NXG most commonly involves the skin (5), NXG involvement of the nervous system is especially rare. Herein, we report a case of NXG of the meninges, brain parenchyma, and spleen. We also summarize its clinical characteristics, imaging manifestations, histopathology, and therapeutic methods.