[Necrobiotic xanthogranuloma in IgG kappa plasmacytoma and Quincke edema].
Necrobiotic xanthogranuloma is a non-X histiocytosis with unknown pathogenesis. It is associated with paraproteinaemia, and in rare cases with multiple myeloma. A decreased level of C1 inhibitor has been found in several cases without clinical manifestations of Quincke oedema. We report on a patient with necrobiotic xanthogranuloma and myeloma, in whom we found a decreased level of C1 inhibitor and recurrent episodes of manifest Quincke oedema. The indirect detection of auto-antibodies against the paraprotein with development of immune complexes is regarded as an explanation for the consumption of the C1 inactivator and the manifestation of Quincke oedema. The possibility of a causal relationship between paraproteinaemia and necrotic xanthogranuloma is discussed.
Hafner
O
O
Hautklinik, Städtischen Krankenhauses Hildesheim.
Witte
T
T
Schmidt
R E
RE
Vakilzadeh
F
F
D000328:Adult; D000799:Angioedema / Q000276:immunology / Q000473:pathology*; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency; D006099:Granuloma / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D008297:Male; D009101:Multiple Myeloma / Q000276:immunology / Q000473:pathology*; D017441:Necrobiotic Disorders / Q000276:immunology / Q000473:pathology*; D009367:Neoplasm Staging; D012867:Skin / Q000276:immunology / Q000473:pathology; D014973:Xanthomatosis / Q000276:immunology / Q000473:pathology*
D003174:Complement C1 Inactivator Proteins; D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains
Nekrobiotisches Xanthogranulom bei IgG-kappa-Plasmozytom und Quincke-Odem.