Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia.
The POEMS syndrome is an infrequently reported multisystem disorder which presents usually as an obscure polyneuropathy, with almost all cases reported in Japan. A 64 yr old caucasian man presented with a 12 mth history of a severe sensorimotor neuropathy in association with dermato-endocrine features. Detection of a monoclonal IgG kappa paraprotein and mixed osteosclerotic/lytic bone lesions consistent with a plasma cell dyscrasia led to diagnosis of the POEMS syndrome. Unique ultrastructural features were present on sural nerve biopsy in addition to the unusual association with monoclonal kappa-light chain. This case illustrates that the POEMS syndrome may also occur in caucasian subjects.
Romas
E
E
Department of Clinical Immunology, St Vincent's Hospital, Melbourne, Victoria.
Storey
E
E
Ayers
M
M
Byrne
E
E
D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000208:ethnology / Q000276:immunology / Q000473:pathology*; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology; D044465:White People
D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains