Solitary plasmacytoma of the skull revealed by a mononeuritis multiplex associated with immune complex vasculitis.
We describe a patient with solitary plasmacytoma of the skull, in whom mononeuritis multiplex was the presenting manifestation. Some features of the POEMS syndrome (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal [M] protein, skin changes), including thrombocytosis, were found. Muscle and nerve biopsies disclosed a small vessel hypersensitivity-type vasculitis and complement-fixing immune complex deposits in vessel walls. Removal of the plasmacytoma resulted in clinical improvement and clearance of the vasculitis and immune complex deposits.
Gherardi
R K
RK
Department of Pathology, (Neuropathology), Hôpital Henri Mondor, Créteil, France.
Amiel
H
H
Martin-Mondiere
C
C
Viard
J P
JP
Salama
J
J
Delaporte
P
P
D000328:Adult; D015922:Complement C1q / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007105:Immune Complex Diseases / Q000276:immunology* / Q000473:pathology; D009132:Muscles / Q000098:blood supply / Q000294:innervation / Q000473:pathology; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009420:Nervous System / Q000098:blood supply / Q000473:pathology; D009422:Nervous System Diseases / Q000276:immunology* / Q000473:pathology; D010954:Plasmacytoma / Q000276:immunology* / Q000473:pathology; D012888:Skull Neoplasms / Q000276:immunology* / Q000473:pathology; D014657:Vasculitis / Q000276:immunology* / Q000473:pathology
D003176:Complement C3; D015922:Complement C1q