Vernal keratoconjunctivitis

Title

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Other Names:

VKC

Categories:

Eye diseases

Summary Summary

Vernal keratoconjunctivitis (VKC) is a chronic, severe allergy that affects the surfaces of the eyes. It most commonly occurs in boys living in warm, dry climates. Attacks associated with VKC are common in the spring (hence the name "vernal") and summer but often reoccur in the winter.^[1]^[2] Signs and symptoms usually begin before 10 years of age and may include hard, cobblestone-like bumps (papillae) on the upper eyelid; sensitivity to light; redness; sticky mucus discharge; and involuntary blinking or spasms of the eyelid (blepharospasm).^[1]^[2] The condition usually subsides at the onset of puberty. It is caused by a hypersensitivity (allergic reaction) to airborne-allergens.^[2] Management focuses on preventing "flare ups" and relieving the symptoms of the condition.^[2]

Last updated: 12/7/2011

Symptoms 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.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormal conjunctiva morphology		0000502
Abnormal sclera morphology		0000591
Allergy		0012393
Corneal neovascularization	New blood vessel formation in cornea	0011496
Lacrimation abnormality	Abnormality of tear production	0000632
Photophobia	Extreme sensitivity of the eyes to light Light hypersensitivity [ more ]	0000613
Pruritus	Itching Itchy skin Skin itching [ more ]	0000989
Punctate keratitis		0011859
30%-79% of people have these symptoms
Scarring		0100699

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Last updated: 2/1/2021

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Treatment Treatment

Management of vernal keratoconjunctivitis (VKC) focuses on preventing allergic attacks as well as relieving the signs and symptoms of the condition. It is often recommended that affected individuals try to avoid the agent that causes the allergy (if possible); wear dark sunglasses in the daytime; avoid dust; and stay inside on hot afternoons. Eye drops that affect the amount of histamine released by immune system cells (called mast cell stabilizers) may be used at the beginning of the season or at the first sign of a "flare-up" to prevent severe symptoms; however, they are not considered effective at relieving symptoms. Topical eye drops are generally preferred as the first source of treatment. Cold compresses, artificial tears, ointments and/or topical antihistamines may help. Non-steroid anti-inflammatory drugs (NSAIDS) may relieve symptoms in moderate cases; topical steroids are typically only used for more severe cases because long-term use can cause glaucoma.^[2]

A few prescription drugs may also be available for the treatment of VKC; these include cromolyn sodium, lodoxamide tromethamine and Levocabastine. Oral administration of montelukast, a drug usually prescribed for asthma, has also been shown to be an effective treatment of VKC.^[2] For more information about these drugs and their availability, individuals should speak with their health care providers.

Last updated: 12/7/2011

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Lodoxamide tromethamine (Brand name: Alomide) - Manufactured by Alcon Laboratories, Inc.
FDA-approved indication: Treatment of ocular disorders referred to by the terms vernal keratoconjunctivitis, vernal conjunctivitis, vernal keratitis.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

Prognosis Prognosis

Vernal keratoconjunctivitis (VKC) generally resolves spontaneously after puberty without any further symptoms or visual complications. However, the development of corneal ulcers (in approximately 9.7% of affected individuals), cataract or glaucoma can potentially cause permanent vision loss.^[3] Beginning treatment immediately after receiving the diagnosis of VKC is very important because the longer an individual has complications from the disease, the greater the chances of developing cataracts or permanent blindness.^[2] It has been reported that the size of the papillae is directly related to the probability of persistence or worsening of symptoms (i.e. the larger the papillae, the worse the prognosis is). It has also been reported that the bulbar forms of VKC have a worse long-term prognosis than the tarsal forms.^[3]

Last updated: 12/7/2011

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Vernal keratoconjunctivitis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations 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

Ocular Immunology and Uveitis Foundation
348 Glen Road
Weston, MA 02493
Telephone: +1-781-647-1431
E-mail: fosters@uveitis.org; ajustus@mersi.com (support)
Website: https://uveitis.org/

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Learn More 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

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.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Vernal keratoconjunctivitis. Click on the link to view a sample search on this topic.

News & Events News & Events

News

Rare Disease Day at NIH 2021
March 1, 2021

GARD Answers GARD Answers

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My son, 9 years old, has been diagnosed with this condition and I am looking for general information on incidence, prognosis and treatment. Thank you. See answer

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References References

Pedram Hamrah, Reza Dana. Vernal keratoconjunctivitis. UpToDate. 2011;
Vernal keratoconjunctivitis. NORD. August 25, 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/562/viewAbstract. Accessed 12/7/2011.
S Bonini, M Coassin, S Aronni and A Lambiase. Vernal keratoconjunctivitis. Eye. 2004; 18:345-351.

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