|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1988, 1989, 1994, 2003, 2011, 2014
NORD is very grateful to 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 a hypersensitivity to airborne-allergens. It usually affects younger members of the population, ages 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.
VKC symptoms include inflammation of the outer membrane of the eye. This causes the eyes to become red and may cause blurred vision. The eyes become sensitive to light and itch intensely. Usually both eyes are affected, and cobblestone-like changes appear in the upper eyelid linings (palpebral conjunctiva). In other cases, a gelatinous nodule may develop in the tissue adjacent to the cornea (limbus). In very severe cases, the corneas may scar (shield ulcers) or clouding of the lens (cataract) may occur, leading to temporarily or permanently reduced vision.
The cause of VKC is a hypersensitivity or allergic reaction of the eyes to airborne allergens. The pathogenesis of ocular allergies is related to a complex exchange of information between tissues through cell-to-cell communications, chemical mediators, cytokines, and adhesion molecules. It is also possible that the neural and endocrine systems may influence ocular allergic responses.
The longer a patient suffers from seasonal VKC, the more likely he or she is to develop the disease chronically. That is why it is important to obtain a swift diagnosis and treat the disease as soon as possible.
Onset of VKC typically occurs between ages 3 and 25 years. The disorder appears to affect more males than females. Usually patients with VKC have a family history of atopic diseases, such as asthma, eczema, or rhinitis. It occurs most often during the spring or summer but up to 60% of patients experience recurrences in the winter periodically. It is more present in climates that are dry and warm.
Symptoms of the following disorder can be similar to those of vernal keratoconjunctivitis. Comparisons may be useful for a differential diagnosis.
Conjunctivitis or "pink eye" is caused by an infection of the outer lining of the eye and eyelids from bacteria or viruses. The eyes become red and irritated with a sandy or burning feeling. The disease may follow a cold or sore throat, and is most common in children. Sticky pus is visible in the eye and can cause the eyelids to stick together. Pink eye is highly contagious
The diagnosis of VKC is usually straightforward and can almost always be diagnosed based on signs and symptoms. However, atypical presentations or patients that experience incomplete forms of VKC may have a more difficult time obtaining a diagnosis. Having asthma or dermatitis also helps support the diagnosis of VKC.
Clinical Testing and Work-Up
Difficult cases of VKC can be diagnosed and treated by conjunctival scraping, demonstrating the presence of infiltrating eosinophils.
VKC usually subsides at the onset of puberty, and treatment is both preventive and therapeutic. To prevent flare-ups, the agent that causes the allergy should be avoided if possible. Wearing dark sunglasses in the daytime, avoiding dust, and not going out on hot afternoons are also recommended. Mast cell stabilizer eye drops can be used at the beginning of the season or the first sign of a flare-up to prevent severe symptoms.
Topical eye drops are generally preferred as the first source of treatment. Cold compresses and artificial tears and ointments also may soothe, lubricate and dilute the antigen. Topical antihistamines producing constriction of the blood vessels and ducts may help. Mast-cell stabilizers may prevent further flare-ups of the disorder or may help control a flare-up, but do little to reduce the symptoms of VKC. Non-steroid anti-inflammatories (NSAIDS) may relieve symptoms in moderate cases but topical steroids should be reserved for more severe cases. Topical steroid preparations are the most effective therapy for moderate to severe forms of VKC, however, their use should be carefully monitored because long-term use can cause glaucoma.
It is important to begin treatment of VKC immediately upon receiving a diagnosis because the longer a patient suffers with the disease, the chances of developing cataracts or permanent blindness increases.
A few prescription drugs are also available for the treatment of VKC, such as the orphan drug cromolyn sodium. Cromolyn has been shown to reduce signs and symptoms of VKC. For more information about cromolyn, please contact:
6111 Ave Royalmount, Suite 100
Phone: (514) 340-9800
The orphan product lodoxamide tromethamine (Alomide Ophthalmic Solution) has also been approved for treatment of vernal keratoconjunctivitis. The product is manufactured by:
6201 South Freeway
Ft. Worth, TX 76134
The orphan drug Levocabastine has also been approved by the FDA for the treatment of this disorder. For more information on this drug, physicians may contact:
500 Iolab Drive
Claremont, CA 91711
Oral administration of montelukast (Singulair), a drug usually prescribed for asthma, has been shown to be an effective treatment of VKC. For more information on Montelukast, please contact Merck:
One Merck Drive
P.O. Box 100
Whitehouse Station, NJ 08889-0100 USA
Monday-Friday 8:30 AM - 5:30 PM ET\
Phone: (800) 444-2080
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
For information about clinical trials sponsored by private sources, contact:
For information about clinical trials conducted in Europe, contact:
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:714-16, 1050.
Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:826-27.
Larson DE, ed. Mayo Clinic Family Health Book. New York, NY: William Morrow and Company, Inc; 1996:542-43.
Bonini S, Coassin M, Aronni S, and Lambiase A. Vernal keratoconjunctivitis. Eye. 2004: 18, 345-351. http://www.nature.com/eye/journal/v18/n4/full/6700675a.html
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.
M-Eye Conditions. University of Michigan, Kellogg Eye Center. Vernal Keratoconjunctivitis. http://www.kellogg.umich.edu/patientcare/conditions/vernal.html Accessed Feb 11, 2014.
Handbook of Ocular Disease Management. Vernal keratoconjunctivitis (VKC).
http://cms.revoptom.com/handbook/oct02_sec2_3.htm Accessed Feb 11, 2014.
Chang-Godinich A. Medscape. Atopic Keratoconjunctivitis. Updated: May 29, 2013. http://emedicine.medscape.com/article/1194480-overview Accessed Feb 11, 2014.
Report last updated: 2014/02/12 00:00:00 GMT+0