title: [Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy].
abstract: BACKGROUND
  Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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
authors.forename: S
authors.initials: S
authors.lastname: Roth
authors.forename: E
authors.initials: E
authors.lastname: Delmont
authors.forename: F
authors.initials: F
authors.lastname: Heinrich
authors.forename: P
authors.initials: P
authors.lastname: Heudier
authors.forename: C
authors.initials: C
authors.lastname: Perrin
authors.forename: R
authors.initials: R
authors.lastname: Kaphan
authors.forename: E
authors.initials: E
authors.lastname: Cua
authors.forename: J G
authors.initials: JG
authors.lastname: Fuzibet
mesh_terms: D000368:Aged; D005260:Female; D006099:Granuloma / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology* / Q000473:pathology; D006801:Humans; D009336:Necrosis; D010265:Paraproteinemias / Q000175:diagnosis* / Q000473:pathology
vernacular_title: La xanthogranulomatose nécrobiotique, une histiocytose non langerhansienne à expression cutanée associée à une gammapathie monoclonale.