Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy

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Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy

RATIONALE Xanthomatosis associated with monoclonal gammopathy includes hyperlipidaemic xanthoma (HX), normolipidaemic xanthoma (NX) and necrobiotic xanthogranuloma (NXG). All three pathologies are characterized by skin or visceral lesions related to cholesterol accumulation, monoclonal immunoglobulin (MIg) and hypocomplementemia. The pathophysiology underlying NXG remains unknown although the i...

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Atypical Necrobiotic Xanthogranuloma Associated with Epidermodysplasia Verruciformis

Necrobiotic xanthogranuloma is a rare, idiopathic, and chronic systemic granulomatous disorder that is usually associated with paraproteinemia of the immunoglobulin (Ig)G kappa (κ) type. It is clinically characterized by yellowish red-to-brown indurated often ulcerating nodules or plaques that usually involve the periorbital regions of the elderly. We report an atypical case of NXG affecting on...

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Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

An 82-year-old woman presented with a 9-month history of multiple, well-defined skin lesions on her neck and upper chest, progressively increasing in size. Histological examination of a skin biopsy showed a regular epidermis. In the dermis, granulomatous changes with central necrobiosis were found which extended focally into the subcutaneous fat. The necrobiotic areas were surrounded by Touton ...

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Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin.

BACKGROUND Necrobiotic xanthogranuloma (NXG) is a rare chronic disorder characterized by firm yellow to red-orange nodules and plaques affecting the face, abdomen, and extremities with the potential for systemic involvement. NXG has a close association with monoclonal gammopathies, and there is a predilection for the development of multiple myeloma. Treatment options are varied due to inconsist...

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Interferon alpha-2a monotherapy for necrobiotic xanthogranuloma.

oids (5, 6). In our patient the titre of circulating anti-ICS antibodies was low, which might indicate a prolonged initial phase of PV before generalized development of bullae. The massive plasma cell in®ltrate seen in the lesional upper dermis may be a factor for the localization of the lesions. Another possibility is that an unknown mechanism prevents the generalization of PV, resulting in th...

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ژورنال

عنوان ژورنال: Journal of Internal Medicine

سال: 2014

ISSN: 0954-6820,1365-2796

DOI: 10.1111/joim.12195