Periocular Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin

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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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Periorbital necrobiotic xanthogranuloma treated successfully with novel multiple myeloma therapy.

A 46-year-old woman with a medical history of a multinodular goiter presented to the department of dermatology with extensive yellow papules and nodules surrounding both eyelids that had developed over the past 3 years. A skin biopsy was done that showed chronic granulomatous dermal inflammation and foreign body giant reaction consistent with juvenile xanthogranuloma after ruling out a ruptured...

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Symptomatic IgG3 deficiency successfully treated with intravenous immunoglobulin therapy.

We report the case of a 35 year old female who presented with an 8 year history of repeated upper respiratory tract infection, lymphadenopathy and sinusitis associated with IgG3 deficiency. Courses of monthly intravenous immunoglobulin therapy (0.4 g/kg) resulted in a dramatic clinical improvement on three different occasions. We suggest that investigation of adults with features of immunosuppr...

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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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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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ژورنال

عنوان ژورنال: Acta Dermato Venereologica

سال: 2017

ISSN: 0001-5555

DOI: 10.2340/00015555-2626