A case of atypical POEMS syndrome without polyneuropathy.
POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) syndrome is a rare hematological disease associated with overproduction of pro-inflammatory cytokines. Under the current nomenclature and diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy is required for diagnosis. We report a 43-year-old Japanese woman with organomegaly, endocrinopathy, M-protein, skin lesions, as well as typical renal lesions and sclerotic bone lesions. Of note, neurological examinations and peripheral nerve conduction tests were normal in this patient. In view of the overwhelming number of otherwise characteristic signs and symptoms, we made a provisional diagnosis of 'atypical POEMS syndrome without polyneuropathy'. If further similar cases are reported in the future, reconsideration of the nomenclature and/or diagnostic criteria for POEMS syndrome may be required.
Morizane
Ryuji
R
Department of Internal Medicine, School of Medicine, Keio University, Tokyo, Japan. morizanr@da2.so-net.ne.jp
Sasamura
Hiroyuki
H
Minakuchi
Hitoshi
H
Takae
Yujiro
Y
Kikuchi
Haruhito
H
Yoshiya
Naomi
N
Hashiguchi
Akinori
A
Konishi
Konosuke
K
Okamoto
Shinichiro
S
Itoh
Hiroshi
H
D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D007122:Immunoelectrophoresis; D008279:Magnetic Resonance Imaging; D016878:POEMS Syndrome / Q000473:pathology* / Q000601:surgery; D011115:Polyneuropathies