[Necrobiotic xanthogranuloma with skin and liver amyloidosis]

Necrobiotic xanthogranuloma is a largely unknown disease. Both the frequency of this disease and the involvement of internal organs have clearly been underestimated until now.

A patient was admitted because of ulcerating, xanthomatous, subcutaneous nodules, scleritis and conjunctivitis. Laboratory studies revealed an elevated erythrocyte sedimentation rate, a monoclonal IgG-lambda protein, a pancytopenia and later a hypocomplementemia. Furthermore, a hepatosplenomegaly and esophageal varices were found. A skin biopsy specimen showed a granulomatous infiltrate consisting of lymphocytes, histiocytes, plasma cells, foam cells as well as Touton and bizarre-appearing foreign-body giant cells and cholesterol clefts typical of necrobiotic xanthogranuloma besides a deposit of amyloid. A liver biopsy sample disclosed an amyloidosis of the parenchyma, too. Neither a therapy with chlorambucil and prednisolone nor with interferon alpha-2a resulted in improvement.

Apart from treatment of skin lesions and ophthalmic manifestations further investigations are necessary because necrobiotic xanthogranuloma can be associated with malignancy and can involve internal viscera like lungs, heart and liver.