POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions.
A rare form of plasma cell dyscrasia characterized by the various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes has been termed POEMS syndrome. The pathogenesis of the multisystemic features of this syndrome remains unclear. Herein is reported a case of POEMS syndrome with striking clinical similarities with scleroderma, and microangiopathic glomerular lesions, as well as diffuse perivascular non-amyloid deposits, which could explain certain features of the syndrome, including peripheral nerve demyelination. It is proposed that a pathogenic role might be played by a non-immunoglobulin vasculotoxic component.
Viard
J P
JP
Service d'Immunologie Clinique, INSERM U25, Hôpital Necker, Paris, France.
Lesavre
P
P
Boitard
C
C
Noel
L H
LH
Roth
A
A
Said
G
G
Bach
J F
JF
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology*; D008214:Lymphocytes / Q000473:pathology; D008854:Microscopy, Electron; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology; D011115:Polyneuropathies / Q000150:complications* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000209:etiology*; D013497:Sural Nerve / Q000473:pathology; D013577:Syndrome