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ORPHA:902Disease
Also called Adult progeria · WS
What it is
Werner syndrome (WS) is a rare inherited syndrome characterized by premature aging with onset in the third decade of life and with cardinal clinical features including bilateral cataracts, short stature, graying and thinning of scalp hair, characteristic skin disorders and premature onset of additional age-related disorders.
Key facts
- Prevalence
- 1-9 / 1 000 000 (Europe)
- Age of onset
- Adolescent, Adult
- Inheritance
- Autosomal recessive
- 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%
15- Abnormal hair whorl
- Abnormality of the voice
- Abnormal thorax morphology
- CataractDiagnostic criterion
- Convex nasal ridge
- Hypogonadism
- Lipoatrophy
- Osteoporosis
- Pili torti
- Premature graying of hairDiagnostic criterion
- Prematurely aged appearance
- Short statureDiagnostic criterion
- Slender build
- Sparse scalp hair
- White forelock
Common30–79%
23- Abnormality of retinal pigmentation
- Abnormal testis morphology
- Aplasia/Hypoplasia of the skin
- Aplasia/Hypoplasia of the testes
- Atherosclerosis
- Chondrocalcinosis
- Congestive heart failure
- Decreased fertility
- HyperkeratosisDiagnostic criterion
- Increased bone mineral density
- Insulin resistance
- Lack of skin elasticityDiagnostic criterion
- LipodystrophyDiagnostic criterion
- Myocardial infarction
- Narrow face
- Pulmonary artery stenosis
- Rocker bottom foot
- Skeletal muscle atrophy
- Skin ulcerDiagnostic criterion
- Small hand
- Subcutaneous calcification
- Telangiectasia of the skin
- Type II diabetes mellitus
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.
Gene
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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