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Start free with EleplanHereditary sensory and autonomic neuropathy type 2
ORPHA:970Disease
Also called Autosomal recessive sensory radicular neuropathy · HSAN2 · Hereditary sensory and autonomic neuropathy type II · Neurogenic acroosteolysis
What it is
A rare hereditary sensory and autonomic neuropathy characterized by profound and universal sensory loss involving large and small fiber nerves.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Infancy, Neonatal
- 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 cortical bone morphology
- Abnormality of epiphysis morphology
- Abnormality of the ankles
- Abnormality of the hip bone
- Abnormality of the knee
- Acroosteolysis (feet)
- Dystrophic fingernails
- Dystrophic toenail
- Hyperhidrosis
- Hyperlordosis
- Osteolysis
- Reduced bone mineral density
- Skeletal muscle atrophy
- Tapered finger
- Wormian bones
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
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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