Hyperhidrosis, Gustatory (HYPRG)

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Disease Ontology 12 DOID:11599 OMIM® 57 144100 MeSH 44 D013547 SNOMED-CT 67 56046002

MedGen 41 C0038994 SNOMED-CT via HPO 68 161857006 263681008 312230002 364538006 415690000 415691001 52613005 more EFO 17 EFO_1000940 UMLS 70 C0038994 C0162473

GARD : ²⁰ Frey's syndrome is a rare, neurological disorder that causes a person to sweat excessively while eating. It most often occurs as a complication of surgery involving the parotid gland (a major salivary gland located below the ear). It may also occur following neck dissection, facelift procedures, or trauma to the area near the parotid gland. Symptoms usually develop within several months of the procedure or trauma, but may develop several years later. The main symptoms include flushing and excessive sweating ( hyperhidrosis ) on the cheek, temple, or behind the ear, when eating or thinking about food ( gustatory sweating ). Some people with Frey's syndrome may experience a burning sensation, itching, or pain around the affected area. The symptoms are usually mild but can be severe, causing significant discomfort or social anxiety. Frey's syndrome is thought to be caused by damage to both the nerves that regulate the sweat glands, and the nerves that regulate the parotid glands. It is believed that the damaged nerves regrow abnormally and connect to the wrong glands. Frey's syndrome is diagnosed based on medical history (e.g. a history of surgery or trauma) and symptoms. The diagnosis can be confirmed with a test called the Minor's starch-iodine test. Treatment, when needed, is focused on controlling the symptoms and often involves injections of botulinum toxin A (Botox) and/or topical antiperspirants. Repeat Botox injections are often needed as symptoms return, which occurs yearly on average. Of note, although Botox injections have been reported to improve symptoms and quality of life, no randomized controlled trials of its use to treat Frey's syndrome have been documented.

MalaCards based summary : Hyperhidrosis, Gustatory, also known as frey syndrome, is related to diabetic neuropathy and peripheral nervous system disease. An important gene associated with Hyperhidrosis, Gustatory is TRPV1 (Transient Receptor Potential Cation Channel Subfamily V Member 1), and among its related pathways/superpathways are Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds and Development Endothelin-1/EDNRA signaling. Affiliated tissues include salivary gland and skin, and related phenotypes are hyperhidrosis and integument

OMIM® : ⁵⁷ Gustatory sweating results from a disruption of the auriculotemporal nerve pathways. Damage to the nerve may cause a misdirected regrowth that results in parasympathetic innervation of sympathetic receptors and, therefore, facial sweating and flushing with gustatory stimulation (summary by Dunbar et al., 2002). (144100) (Updated 05-Apr-2021)

Wikipedia : ⁷³ Gustatory hyperhidrosis is excessive sweating classified under focal hyperhidrosis, that is, it is... more...

Clinical features from OMIM®:

144100 (Updated 05-Apr-2021)

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