[Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy].
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.
Roth
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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
Delmont
E
E
Heinrich
F
F
Heudier
P
P
Perrin
C
C
Kaphan
R
R
Cua
E
E
Fuzibet
J G
JG
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
La xanthogranulomatose nécrobiotique, une histiocytose non langerhansienne à expression cutanée associée à une gammapathie monoclonale.