[POEMS-Syndrome--unusual manifestation with bilateral Charcot's Joint].
METHODS
A 45-year-old patient with no significant past medical history was admitted 8 weeks after minor foot trauma with injury of the left ankle joint with massive destructive arthropathy. On admission the arch of both feet was flattened and both feet showed massive painless soft tissue swelling. Due to loss of muscle strength elevation of feet and toes was reduced and there was a "stocking-like" loss of sensation of the lower extremity. The gait was ataxic. No other abnormalities were detected besides hepatomegaly and hyperhidrosis.


METHODS
Advanced polyneuropathy was confirmed by nerve conduction study. Laboratory tests revealed paraproteinaemia type IgG-lambda. Bone marrow biopsy, bone scan and skeletal survey did not provide additional information. Sonography confirmed hepatosplenomegaly, endocrinological work up showed impaired glucose tolerance and lowered testosterone levels. Based on these findings the diagnosis of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) was established.


METHODS
Plasma exchange was unsuccessful and due to worsening of the symptoms long term treatment with cyclophosphamide was started. Furthermore the patient received NSAIDs for symptomatic relief and orthopedic shoes. One year after initial presentation the patient continues to walk.


CONCLUSIONS
Usually neurogenic arthropathy is associated with diabetes mellitus, syringomyelia or with infections. Our report illustrates that POEMS syndrome might present with Charcot arthritis.
Mäurer
M
M
Neurologische Klinik und Poliklinik, Bayerischen Julius-Maximilians Universität Würzburg.
Sommer
C
C
D016512:Ankle Injuries / Q000150:complications; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001177:Arthropathy, Neurogenic / Q000209:etiology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D009765:Obesity / Q000150:complications; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D010951:Plasma Exchange
D000894:Anti-Inflammatory Agents, Non-Steroidal; D003520:Cyclophosphamide