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Start free with EleplanPOEMS syndrome
ORPHA:2905Disease
Also called Crow-Fukase syndrome · Osteosclerotic myeloma · PEP syndrome · Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome · Takatsuki syndrome
What it is
POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels.
Key facts
- Prevalence
- 1-9 / 1 000 000 (Japan)
- Age of onset
- Adult, Elderly
- Inheritance
- Unknown
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
6- Abnormality of skin physiologyDiagnostic criterion
- Abnormality of the endocrine systemDiagnostic criterion
- Hypogonadism
- Increased circulating immunoglobulin concentration
- Lymphoproliferative disorderDiagnostic criterion
- VisceromegalyDiagnostic criterion
Common30–79%
33- Areflexia
- AscitesDiagnostic criterion
- Clubbing of fingers
- Diabetes mellitus
- EdemaDiagnostic criterion
- Elevated vascular endothelial growth factor levelDiagnostic criterion
- Erectile dysfunction
- Fatigue
- Gynecomastia
- HemangiomaDiagnostic criterion
- HepatomegalyDiagnostic criterion
- Hyperpigmentation of the skinDiagnostic criterion
- HypertrichosisDiagnostic criterion
- Hypothyroidism
- Leukonychia
- LymphadenopathyDiagnostic criterion
- Metaphyseal sclerosisDiagnostic criterion
- Muscle weakness
- Pain
- PapilledemaDiagnostic criterion
- Paresthesia
- Pericardial effusionDiagnostic criterion
- Pleural effusionDiagnostic criterion
- Primary adrenal insufficiency
- Pulmonary arterial hypertension
- Sclerosis of foot boneDiagnostic criterion
- Sclerosis of hand boneDiagnostic criterion
- Sclerosis of skull baseDiagnostic criterion
- Sclerotic vertebral endplatesDiagnostic criterion
- SplenomegalyDiagnostic criterion
- Thickened skin
- ThrombocytosisDiagnostic criterion
- Weight loss
Sometimes5–29%
11- AcrocyanosisDiagnostic criterion
- Arterial thrombosis
- Glomeruloid hemangioma
- Hyperesthesia
- Increased circulating prolactin concentration
- Lipodystrophy
- PlethoraDiagnostic criterion
- PolycythemiaDiagnostic criterion
and 3 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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