title: A case of atypical POEMS syndrome without polyneuropathy.
abstract: 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.
authors.affiliation: Department of Internal Medicine, School of Medicine, Keio University, Tokyo, Japan. morizanr@da2.so-net.ne.jp
authors.forename: Ryuji
authors.initials: R
authors.lastname: Morizane
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sasamura
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Minakuchi
authors.forename: Yujiro
authors.initials: Y
authors.lastname: Takae
authors.forename: Haruhito
authors.initials: H
authors.lastname: Kikuchi
authors.forename: Naomi
authors.initials: N
authors.lastname: Yoshiya
authors.forename: Akinori
authors.initials: A
authors.lastname: Hashiguchi
authors.forename: Konosuke
authors.initials: K
authors.lastname: Konishi
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Okamoto
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Itoh
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D007122:Immunoelectrophoresis; D008279:Magnetic Resonance Imaging; D016878:POEMS Syndrome / Q000473:pathology* / Q000601:surgery; D011115:Polyneuropathies