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POEMS syndrome
Other Resources UpToDate PubMed

POEMS syndrome

Contributors: Nneka I. Comfere MD, Jeffrey M. Cohen MD, Susan Burgin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


POEMS syndrome is a multisystem disorder associated with underlying plasma cell dyscrasia. POEMS is a syndrome of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes and sclerotic bone changes.

While the etiology of POEMS syndrome is not known, it is attributed to chronic inflammation (cytokine overproduction) resulting in microangiopathy, edema, increased vascular permeability, and neovascularization. Polyneuropathy, pulmonary hypertension, leukocytosis, and thrombocytosis are possible consequences.

POEMS syndrome should be considered in patients with polyneuropathy and evidence of a monoclonal plasma cell disorder. It is a rare disorder and affects patients predominantly in their fifth and sixth decades of life, with a median age of 51 years and a slight male predominance. While all patients with POEMS syndrome will have polyneuropathy and monoclonal cell disorders, and the vast majority will have osteosclerotic bone lesions, only about two-thirds will have associated skin changes and endocrinopathy. About one-half will have concurrent organomegaly, and about one-third will have fatigue and weight loss. Castleman disease is seen in about 15% of cases.

Criteria used for diagnosis include the following.

Mandatory major criteria:
  • Polyneuropathy that is frequently demyelinating (100%)
  • Monoclonal plasma cell proliferation (100%) – usually immunoglobulin G (IgG), almost always lambda chain
Other major criteria include:
  • Castleman disease (angiofollicular lymph node hyperplasia)
  • Marked elevation of serum vascular endothelial growth factor (VEGF) levels
  • Extravascular volume overload (ascites, pleural effusions)
  • Endocrinopathy that may have affected several glands (67%-84%)
  • Bone lesions – usually sclerotic (27%-97%)
  • Multiple skin changes (detailed below) (68%-89%)
  • Papilledema (29%-64%)
  • Thrombocytosis (54%-88%)


D47.9 – Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified

79268002 – POEMS syndrome

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Differential Diagnosis & Pitfalls

  • Multiple myeloma
  • Monoclonal gammopathy of undetermined significance (MGUS)
  • Waldenström macroglobulinemia
  • Solitary plasmacytoma of bone
  • AL amyloidosis
  • Cryoglobulinemia
  • Chronic inflammatory demyelinating polyneuropathy
  • Cushing syndrome
Differential diagnosis of hyperpigmentation and sclerotic skin:
  • Scleroderma
  • Porphyria cutanea tarda
Differential diagnosis of sclerodermoid plaques:
  • Morphea
  • Scleroderma, CREST syndrome
  • Morpheaform Sarcoidosis
  • Porphyria cutanea tarda
  • AL amyloidosis – lower extremities
  • Chronic graft-versus-host disease – sclerodermoid variant
  • Scleromyxedema
  • Sclerotic variant of Pretibial myxedema
  • Anticancer Drug-induced sclerodermoid reactions (bleomycin, taxanes, gemcitabine)
  • Carcinoid syndrome – lower extremities
  • Dermatofibrosarcoma protuberans (DFSP)
  • Leprosy
  • Mast cell leukemia
  • Lobomycosis
  • Rothmund-Thomson syndrome
  • Severe combined immunodeficiency (SCID)
  • Progeria
  • Werner syndrome
  • Phenylketonuria
See also the differential diagnoses of Glomeruloid hemangioma and Hypertrichosis.

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Last Reviewed:03/24/2021
Last Updated:01/20/2022
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POEMS syndrome
A medical illustration showing key findings of POEMS syndrome (Systemic) : Fatigue, Hepatosplenomegaly, Ascites, Lymphadenopathy, Papilledema, Pleural effusion
Clinical image of POEMS syndrome - imageId=6555944. Click to open in gallery.  caption: 'Smooth red papules and nodules scattered on the anterior trunk.'
Smooth red papules and nodules scattered on the anterior trunk.
Copyright © 2024 VisualDx®. All rights reserved.