Last updated:
8/13/2025
Years published: 1988, 1989, 1994, 2003, 2011, 2014, 2017, 2025
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders and Stefano Bonini, MD, Professor and Chairman, Department of Ophthalmology, University of Rome Campus BioMedico, for assistance in the preparation of this report.
Vernal keratoconjunctivitis (VKC) is a chronic, non-contagious allergic disorder with seasonal recurrences usually appearing during the spring or warm weather. VKC is caused by hypersensitivity to airborne allergens. It usually affects people age 3-25 and most patients are males. Major symptoms include itching, sensitivity to light (photophobia) and redness. Signs consist of inflammation of the mucous membrane lining the inside of the eyelid (conjunctiva) and the outer coat of the eyeball (sclera); hard, cobblestone-like bumps (papillae) on the upper eyelid and stringy or mucous discharge.
Treatment includes some preventive measures and several medications.
VKC symptoms include swelling of the outer membrane of the eye (conjunctiva), particularly the upper eyelid lining (palpebral conjunctiva) and the area around the cornea (limbus). This inflammation causes the eyes to become red (ocular hyperemia), intensely itchy, sensitive to light (photophobia) and may produce a gritty or foreign body sensation. In many people, there is also excessive tearing, thick mucus discharge and swelling of the eyelid tissue, which can lead to a drooping upper eyelid (pseudoptosis). Pain may occur when the cornea is involved. Usually both eyes are affected. Based on the pattern of inflammation, VKC is classified into three types:
Although VKC usually resolves after puberty, it can lead to severe visual impairments if the therapy is not adequate. In more severe cases, complications may develop. These include damage to the cornea such as punctate epithelial keratitis (small surface erosions), shield ulcers (shallow, inflammatory corneal ulcers), or permanent scarring. In some people, gelatinous swellings may form near the limbus. If left untreated, VKC can lead to serious vision problems including blurred vision, a condition where the cornea or lens of the eye has an irregular shape (astigmatism) causing blurred or distorted vision both at near and far distances, thinning and bulging of the cornea (keratoconus), clouding of the lens (cataracts) or even corneal perforation.
While the exact cause of vernal keratoconjunctivitis (VKC) is still not fully understood, it appears to involve a mix of genetic, environmental, immune and hormonal factors.
VKC is a complex eye condition once thought to be caused only by a type 1 allergy where the immune system overreacts to things like pollen by producing IgE antibodies. These trigger mast cells to release chemicals like histamine, causing redness, itching and tearing.
However, many people with VKC don’t show signs of allergies, which is why standard treatments like antihistamines often don’t help. VKC also involves type 2 helper T-cells (Th2 cells) that release inflammatory chemicals such as IL-4, IL-13, and TNF-α, leading to tissue thickening and scarring.
Eosinophils, another immune cell, also play a role by releasing toxic proteins that damage the cornea and increase inflammation.
Over time, VKC can become chronic, lasting year-round and potentially causing lasting eye damage if not properly managed.
Vernal keratoconjunctivitis (VKC) is a rare ocular disease in the developed world but its prevalence varies widely by region. It is most common in warm, dry, allergen-rich climates such as central Africa, India and South America. Studies in Ethiopia and Rwanda show significantly higher prevalence among children (ranging from 4.0% to 11.1%), with risk factors including male sex, dust exposure, use of firewood or kerosene, contact with animals and allergic history.
In 2024, an analysis of data from a large health care claims database in the U.S. indicated that the prevalence of VKC among individuals <18 years of age was 1.24 per 10,000 people.
VKC is usually diagnosed through a detailed eye exam and by recognizing the most important symptoms such as eye itching, redness, light sensitivity and thick discharge. During the exam, doctors may see large bumps under the eyelid (called papillae), swelling around the cornea (limbal infiltration) and small white dots (Trantas dots). In more severe cases, the clear front surface of the eye (cornea) may be damaged, showing signs like surface irritation or ulcers, which can be seen using a special dye during the exam. Doctors may also rate severity as mild, moderate, severe, or blinding based on symptoms and eye findings.
When the diagnosis is uncertain, doctors may do additional tests like gently collecting cells from the surface of the eye (conjunctival scraping or impression cytology) or testing tear samples. These can help detect specific immune cells and substances like eosinophils, histamine and other inflammation markers linked to VKC. Some people with VKC also have low vitamin D levels, especially because light sensitivity can limit sun exposure. Monitoring and improving vitamin D levels may support overall health and help manage VKC more effectively.
Treatment
VKC is a chronic allergic eye condition that often improves or disappears as children reach puberty. Until then, treatment focuses on relieving symptoms, preventing flare-ups and protecting long-term eye health. Important measures include avoiding allergens like pollen and dust, staying out of direct sunlight during hot afternoons, wearing sunglasses outdoors, using cold compresses and rinsing the face and hands regularly. Artificial tears can also help wash allergens off the eyes and soothe irritation.
Several medications are used to manage VKC. The first options usually include antihistamine eye drops, mast cell stabilizers and non-steroidal anti-inflammatory drugs (NSAIDs), which help reduce itching and swelling. Starting these medications early in the allergy season or at the first sign of symptoms can help prevent more severe flare-ups. For moderate to severe cases, steroid eye drops are the most effective but must be used carefully, as long-term use can cause cataracts or glaucoma. For safer, long-term control, doctors may prescribe immunomodulatory drops like cyclosporine or tacrolimus, which calm the immune system and reduce the need for steroids.
Several VKC treatments are FDA-approved, including cromolyn sodium (Opticrom), lodoxamide tromethamine (Alomide) and levocabastine (Livostin). In 2021, the FDA also approved cyclosporine ophthalmic emulsion (Verkazia) for children and adults with VKC. Additionally, montelukast (Singulair), a medication commonly used for asthma, has shown benefits when taken by mouth to help control VKC symptoms.
Early and consistent treatment is essential. Left untreated, VKC can lead to serious complications like shield ulcers, corneal scarring, or even vision loss. In very rare and severe cases, surgery may be needed to repair eye damage. Fortunately, most people respond well to medical treatments. For difficult cases, newer therapies like monoclonal antibodies (such as omalizumab or dupilumab) are emerging and may offer hope when other treatments haven’t worked. VKC is usually managed step-by-step, starting with simple measures and moving to stronger therapies as needed.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact: www.centerwatch.com
For information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
JOURNAL ARTICLES
Ali A, Bielory L, Dotchin S, Hamel P, Strube YNJ, Koo EB. Management of vernal keratoconjunctivitis: Navigating a changing treatment landscape. Surv Ophthalmol. 2024;69(2):265-278. doi:10.1016/j.survophthal.2023.10.008
Bruschi G, Ghiglioni DG, Cozzi L, Osnaghi S, Viola F, Marchisio P. Vernal Keratoconjunctivitis: A Systematic Review. Clin Rev Allergy Immunol. 2023;65(2):277-329. doi:10.1007/s12016-023-08970-4
Bonini S, Coassin M, Aronni S, Lambiase A. Vernal keratoconjunctivitis. Eye (Lond). 2004;18(4):345-351. doi:10.1038/sj.eye.6700675
Leonardi A, Secchi AG. Vernal keratoconjunctivitis. Int Ophthalmol Clin. 2003;43:41-58.
Bielory L, Mongia A. Current Opinion of immunotherapy for ocular allergy. Curr Opin Allergy Clin Immunol. 2002;2:447-52.
Bielory L Kempuraj D, Theoharides T. Topical immunopharmacology of ocular allergies. Curr Opin Allergy Clin Immunol. 2002;2:435-45.
Trocme SD, Sra KK. Spectrum of ocular allergy. Curr Opin Allergy Clin Immunol. 2002;2:423-27.
Friedlander MH. Conjunctival provocation testing: overview of recent clinical trials in ocular allergy.. Curr Opin Allergy Clin Immunol. 2002;2:413-17.
INTERNET
Kim BS. Medscape. Atopic Dermatitis. Last Update December 20, 2024. https://emedicine.medscape.com/article/1194480-overview Accessed August 4, 2025.
NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.
NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.
The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).
View reportOrphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.
View reportPlease complete this form to access the requested resource.
