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Necrobiotic xanthogranuloma

Courtney Schadt, MD
Eric Jacobsen, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Necrobiotic xanthogranuloma (NXG) is a form of non-Langerhans histiocytosis characterized by the development of red-brown, violaceous, or yellowish cutaneous papules and nodules that evolve to form infiltrated plaques. The periorbital skin is the most common site for NXG. Extracutaneous involvement of ocular, respiratory, cardiac, or other tissues may also occur in patients with NXG.

Most patients with NXG have an associated monoclonal gammopathy. Other hematologic disorders, including hematologic malignancies, also may occur in association with NXG.

The treatment of NXG is challenging and data on therapeutic options are limited, making the best approach to treatment unclear. Alkylating agents, such as chlorambucil, are commonly used treatments.

The clinical manifestations, diagnosis, and management of NXG will be reviewed here.


In 1980, Kossard and Winkelmann described eight patients with multiple violaceous, atrophic plaques and nodules on the face (particularly periorbital area), skin flexures, and trunk with associated paraproteinemia [1,2]. They named this condition necrobiotic xanthogranuloma based on the distinctive histologic finding of xanthogranulomas with zones of necrobiosis. (See 'Histopathology' below.)

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Literature review current through: Nov 2017. | This topic last updated: Nov 20, 2017.
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