Your browser version may not work well with NCBI's Web applications. More information here...
1: Rev Med Interne. 2002 May;23(5):460-4.Links

[Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy]

[Article in French]

Service de médecine interne, hôpital l'Archet 1, CHU, 151, route de Saint-Antoine de Ginestière, BP 3079, 06202 Nice, France. fuzibet.jg@chu-nice.fr

INTRODUCTION: Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases. EXEGESIS: A 70-year-old patient was affected by a monoclonal gammopathy. She presented with a diplopia related to a retro-orbital tumor. The biopsy showed inflammatory lesions. Five years later, inflammatory xanthomatous skin lesions appeared. Biopsy specimens gave the diagnosis of necrobiotic xanthogranuloma. A 70-year-old woman was referred for inflammatory cutaneous lesions. Clinical, biological investigations and skin biopsies led to the diagnosis of cutaneous sarcoidosis associated with monoclonal gammopathy. Four years later, she developed a nephrotic syndrome. New skin biopsy specimens showed a necrobiotic xanthogranuloma. CONCLUSION: Necrobiotic xanthogranuloma is a systemic disease. It is a rare non-Langerhans cell histiocytosis characterized by frequent cutaneous and ophthalmologic lesions and associated with monoclonal gammopathy. To our knowledge, retro-orbital involvement has never been reported in necrobiotic xanthogranuloma. Treatment is difficult.

PMID: 12064218 [PubMed - indexed for MEDLINE]