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Start free with EleplanInsulin-resistance syndrome type B
ORPHA:2298Disease
What it is
A rare genetic disease that belongs to the group of extreme insulin-resistance syndromes and is due to autoantibodies directed against insulin receptor.
Key facts
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Very common80–99%
14- Abnormal circulating fatty-acid concentration
- Abnormal oral glucose tolerance
- Acanthosis nigricans
- Autoimmunity
- Elevated erythrocyte sedimentation rate
- Fasting hyperinsulinemia
- Glucose intolerance
- Hyperglycemia
- Hyperinsulinemia
- Hypotriglyceridemia
- Insulin resistance
- Postprandial hyperglycemia
- Systemic lupus erythematosus
- Weight loss
Common30–79%
19- Abnormal circulating lipid concentration
- Abnormality of body weight
- Abnormality of circulating leptin level
- Antinuclear antibody positivity
- Decreased body weight
- Decreased serum complement factor B
- Enlarged ovaries
- Enlarged polycystic ovaries
- Fatigue
- Glycosuria
- Hirsutism
- Hypoalbuminemia
- Increased serum testosterone level
- Insulin-resistant diabetes mellitus
- Leukopenia
- Nephritis
- Polycystic ovaries
- Proteinuria
- 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.
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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