Rare Pulmonology News

Disease Profile

Frey’s syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Hyperhidrosis gustatory; Gustatory sweating; Auriculotemporal nerve syndrome;

Summary

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.[1][2] Symptoms usually develop within several months of the procedure or trauma, but may develop several years later.[1][2] 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).[1][2] Some people with Frey’s syndrome may experience a burning sensation, itching, or pain around the affected area.[1] The symptoms are usually mild but can be severe, causing significant discomfort or social anxiety.[1][2]

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.[2] It is believed that the damaged nerves regrow abnormally and connect to the wrong glands.[2] 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.[1]

Treatment, when needed, is focused on controlling the symptoms and often involves injections of botulinum toxin A (Botox) and/or topical antiperspirants.[1] Repeat Botox injections are often needed as symptoms return, which occurs yearly on average.[1][2] 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.[2]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance
0000006
Hyperhidrosis
Excessive sweating
Increased sweating
Profuse sweating
Sweating
Sweating profusely
Sweating, increased

[ more ]

0000975

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

      In-Depth Information

      • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Frey's syndrome. Click on the link to view a sample search on this topic.

        References

        1. Motz KM, Kim YJ. Auriculotemporal Syndrome (Frey Syndrome). Otolaryngol Clin North Am. April, 2016; 49(2):501-509. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5457802/.
        2. Frey's syndrome. National Organization for Rare Disorders (NORD). 2017; https://rarediseases.org/rare-diseases/frey-syndrome/.
        3. Jansen S, Jerowski M, Ludwig L, Fischer-Krall E, Beutner D, Grosheva M. Botulinum toxin therapy in Frey's syndrome: a retrospective study of 440 treatments in 100 patients. Clinical Otolaryngology. April, 2017; 42(2):295-300. https://www.ncbi.nlm.nih.gov/pubmed/27513469.
        4. Li C, Wu F, Zhang Q, Gao Q, Shi Z, Li L. Interventions for the treatment of Frey's syndrome. Cochrane Database Syst Rev. March 17, 2015; 3:https://www.ncbi.nlm.nih.gov/pubmed/25781421.