Preferred Label : Necrobiotic xanthogranuloma;
ICD-11 definition : Necrobiotic xanthogranuloma (NXG) is a rare multisystem histiocytic disease involving
the skin in which widespread infiltrated xanthomatous nodules and plaques are associated
in the majority of cases with paraproteinaemia and a variety of underlying lymphoproliferative
disorders including myeloma. NXG differs from diffuse normolipidaemic plane xanthomatosis
in that the skin lesions are palpably infiltrated and elevated rather than flat and
diffuse and in that necrobiosis and Touton giant cells are present histologically.;
Origin ID : 2138563851;
Automatic exact mappings (from CISMeF team)
Necrobiotic xanthogranuloma (NXG) is a rare multisystem histiocytic disease involving
the skin in which widespread infiltrated xanthomatous nodules and plaques are associated
in the majority of cases with paraproteinaemia and a variety of underlying lymphoproliferative
disorders including myeloma. NXG differs from diffuse normolipidaemic plane xanthomatosis
in that the skin lesions are palpably infiltrated and elevated rather than flat and
diffuse and in that necrobiosis and Touton giant cells are present histologically.
