Necrobiotic xanthogranuloma with cutaneous and cerebral manifestations

Abstract

Necrobiotic xanthogranuloma (NXG) is a rare inflammatory histiocytic disease of the skin. Xanthogranuloma of the central nervous system is rare and few cases have been reported. To the authors' knowledge, there has been no previously reported case of NXG in which an intracranial lesion was found. This 52-year-old man, in whom NXG with all its cutaneous manifestations had been diagnosed, presented with three episodes of generalized tonic-clonic seizures. A contrast-enhanced computerized tomography scan of his brain revealed a bifrontal, dura-based mass lesion. The lesion was excised and reported to be an NXG that was similar, but not identical to the skin lesions. The patient was placed on a regimen of antiepileptic drug and chlorambucil after surgery.

A 7-Year History of Necrobiotic Xanthogranuloma following Asymptomatic Multiple Myeloma: A Case Report

“…Chemotherapy is the most frequently used treatment and generally effective in modifying a disease course. Chlorambucil appears to be the most effective treatment for patients with extensive cutaneous lesions [15–17]. Other systemic agents have been employed with some improvement including systemic steroid [18, 19], chlorambucil plus systemic corticosteroids, [7] cyclophosphamide, [20] melphalan, [21, 22] melphalan plus systemic corticosteroids, [23–25] azathioprine plus systemic corticosteroids, [26] thalidomide [27], and interferon- α 2b [7, 28].…”

Necrobiotic xanthogranuloma

“…2). The granulomas are usually band‐like, 35 but can also have a stellate 42 or nodular 43 shape. The granulomas are composed of foamy histiocytes, lymphocytes, foreign body‐type multinucleated giant cells, and Touton giant cells alternating with foci of collagen necrobiosis (Fig.…”

Local Corticosteroid Treatment of Eyelid and Orbital Xanthogranuloma

“…3 Erdheim-Chester disease typically involves the posterior portion of the orbit and is associated with widespread systemic disease that results in death due to infiltration of vital organs. 5,6 This disorder, 7,8 like NXG 9 and AXG, 10 has been routinely treated with combinations of systemic corticosteroids, cytotoxic agents, and radiotherapy. However, NXG and AXG tend to affect the eyelids and anterior orbital tissue.…”