ICD-11 classes
14 Diseases of the skin
Sensory and psychological disorders affecting the skin
Neurological conditions affecting the skin
8C21 — Hereditary sensory or autonomic neuropathy
8C21.1 — Hereditary sensory and autonomic neuropathy type III
ICD-11 8C21.1 — Hereditary sensory and autonomic neuropathy type III
Hereditary sensory and autonomic neuropathy, type 3 (HSAN3) is an autosomal recessive disorder seen primarily in Ashkenazi Jewish children caused by a mutation in the I-kappa B kinase associated protein. It is characterised by sensory dysfunction and severe impairment of the autonomic nervous system activity, resulting in multisystem dysfunction. Symptoms can include insensitivity to pain and temperature, intact visceral pain, alacrima, hypoactive corneal and tendon reflexes and absence of lingual fungiform papillae.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: