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ORPHA:117Disease
What it is
A rare, chronic, relapsing, multisystemic vasculitis characterized by mucocutaneous lesions, as well as articular, vascular, ocular and central nervous system manifestations.
Key facts
- Prevalence
- 1-9 / 100 000 (United States)
- Age of onset
- Adolescent, Adult, Childhood
- Inheritance
- Multigenic/multifactorial
- 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%
14Common30–79%
21- Abdominal pain
- Abnormal blistering of the skin
- Acne
- Arthralgia
- Confusion
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Erythema nodosum
- Gait disturbance
- Gastrointestinal hemorrhage
- Genital ulcers
- Headache
- Hemiparesis
- Immunologic hypersensitivity
- Increased inflammatory response
- Nongranulomatous uveitis
- Panuveitis
- Positive pathergy test
- Pustule
- Superficial thrombophlebitis
- Venous thrombosis
Sometimes5–29%
48- AA amyloidosis
- Abnormal myocardium morphology
- Abnormal pyramidal sign
- Anorexia
- Aortic regurgitation
- Arterial thrombosis
- Ataxia
- Atypical behavior
and 40 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.
Genes
ICD-10 codes
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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