Atopic Keratoconjunctivitis

Atopic keratoconjunctivitis is a rare but potentially blinding eye disease that causes chronic inflammation and severe itching, affecting people with a history of allergic conditions like eczema or asthma.

Table of contents

• What is atopic keratoconjunctivitis?
• Who gets this condition?
• Signs and symptoms
• What causes atopic keratoconjunctivitis?
• Risk factors
• How is it diagnosed?
• Possible complications
• Treatment options
• Living with the condition

AKC

• Eyes
• Eyelids
• Cornea
• Conjunctiva

What is atopic keratoconjunctivitis?

Atopic keratoconjunctivitis is a chronic inflammatory disease of the eye that causes ongoing swelling and irritation on the surface of the eyes, the eyelids, and the skin around the eyes^[1][2]. The condition is rare but serious, and without proper treatment, it can lead to permanent vision loss.

The disease gets its name from two parts of the eye that become inflamed. Keratitis refers to inflammation of the cornea, which is the clear window at the front of your eye. Conjunctivitis refers to inflammation of the conjunctiva, the clear film that covers the white part of your eye^[5]. When both are inflamed at the same time, it means the entire surface of the eyeball is affected.

Who gets this condition?

Atopic keratoconjunctivitis is a rare condition that occurs in less than 1 to 8 percent of the population^[16]. The disease most commonly begins when a person is in their late teens or early twenties. The condition reaches its peak between the ages of 30 and 50 years^[1][5].

Men are more likely to develop atopic keratoconjunctivitis than women^[2]. The condition affects both eyes and is chronic, meaning it lasts for a long time and comes and goes with little connection to seasons, unlike some other types of allergic eye disease^[1].

Almost all people with this condition have other allergic health problems. About 95 percent of people with atopic keratoconjunctivitis also have atopic dermatitis, which is a type of eczema that causes dry, itchy, and red skin. Additionally, 87 percent of people with this eye condition also have asthma^[1].

Signs and symptoms

The main symptom of atopic keratoconjunctivitis is intense itching of the eyes^[1][2]. This itching affects the surface of the eyes, the eyelids, and the skin around the eyes. Many people feel a strong urge to rub their eyes, which can make the condition worse.

People with this condition commonly experience additional eye symptoms, including:

• Redness or pink appearance of the eyes
• Burning sensation in the eyes
• Excessive tearing or watery eyes
• Mucoid or white discharge from the eyes
• Increased sensitivity to light
• Blurred vision
• Foreign body sensation, as if something is in the eye^[2][4][5]

The eyelids themselves show visible changes. They may become thick, crusty, and develop cracks. The skin of the eyelids often shows hyperpigmentation, meaning it becomes darker than normal, along with redness and swelling^[1][5]. In severe cases, the lower eyelid may turn outward, a condition called ectropion, and people may lose eyelashes, known as madarosis^[1].

Eczema around the eyes is almost always present in people with atopic keratoconjunctivitis^[1]. Symptoms often persist throughout the year, though they may become worse during winter months^[5].

What causes atopic keratoconjunctivitis?

Atopic keratoconjunctivitis results from a genetic condition called atopy^[2][5]. Atopy is the inherited tendency to develop allergic diseases. It causes the immune system to overreact to common substances in the environment that are normally harmless.

In people with atopy, the body produces excessive amounts of antibodies in response to allergens. These antibodies are proteins that the immune system makes to fight what it perceives as threats. This abnormal allergic response leads to inflammation in the eyelids and on the surface of the eyes, which causes the symptoms of atopic keratoconjunctivitis^[5].

The disease involves a combination of different types of immune reactions. Scientists have found that it includes both immediate allergic reactions mediated by a type of antibody called IgE, and delayed reactions involving immune cells^[4]. People with this condition also have decreased tear production, which results in signs and symptoms of dry eye and increased inflammation of the eye surface^[16].

Risk factors

Several factors increase a person’s chances of developing atopic keratoconjunctivitis. The most significant risk factor is having a personal history of atopic dermatitis, a chronic skin condition that causes eczema^[5]. People with asthma are also at higher risk.

A family history of atopic diseases increases the likelihood of developing this eye condition^[1][5]. If parents or siblings have conditions like eczema, asthma, hay fever, or food allergies, the risk is elevated.

Additional risk factors include:

• Food and environmental allergies or sensitivities that began at a young age
• Living in densely populated cities with increased exposure to pollutants
• Exposure to tobacco smoke
• Antibiotic use during childhood
• Obesity^[16]

People with an eyelid condition caused by staphylococcal bacteria also have an increased risk^[5]. Certain allergens in the environment may worsen symptoms once the condition develops.

How is it diagnosed?

Doctors begin diagnosing atopic keratoconjunctivitis by asking detailed questions about a person’s medical history^[5]. They look for evidence of asthma or atopic dermatitis, as these conditions are present in most people with this eye disease. The doctor also asks about family history of allergic conditions.

The next step involves asking about symptoms and conducting a thorough examination of the eyes and eyelids^[5]. During this examination, the doctor uses special instruments to look at the surface of the eyes, the inside of the eyelids, and the cornea. They check for characteristic signs such as thickened eyelids, inflammation of the conjunctiva, and changes to the cornea.

Because the symptoms can be similar to other eye conditions, doctors may need to rule out other possible causes. Conditions that might be confused with atopic keratoconjunctivitis include vernal keratoconjunctivitis, seasonal allergic conjunctivitis, giant papillary conjunctivitis, and infectious conjunctivitis^[5].

In some cases, additional tests may be performed. Blood tests can measure levels of IgE antibodies, which helps confirm the presence of an atopic condition, though this cannot specifically diagnose atopic keratoconjunctivitis^[16]. A doctor may also take a small sample from the inside of the eyelid using a brush to examine inflammatory cells under a microscope^[16].

Possible complications

Atopic keratoconjunctivitis is considered a potentially blinding disease^[4]. Without proper treatment, the condition can lead to serious complications that permanently damage vision.

The chronic inflammation causes the conjunctiva to develop scar tissue. This can lead to symblepharon, where the conjunctiva sticks to itself, and shortening of the space under the eyelids^[1]. In advanced cases, the conjunctiva can grow over the cornea, a condition called conjunctivalization, which can block vision^[1].

The cornea itself can develop several problems. These include persistent damage to the outer layer of cells, scarring, growth of abnormal blood vessels into the normally clear cornea, and deposits of fatty substances^[4]. The cornea may also develop ulcers, which are open sores that can lead to serious complications^[16].

People with atopic keratoconjunctivitis who repeatedly rub their itchy eyes risk developing keratoconus, a condition where the cornea becomes thin and cone-shaped^[16]. The chronic inflammation and treatments can also lead to the development of cataracts, particularly a type called posterior subcapsular cataract that forms at the back of the eye’s lens^[1].

These complications develop quickly in some patients, especially younger people with more severe disease^[16]. This makes regular monitoring by eye care professionals essential for anyone with this condition.

Treatment options

Treatment for atopic keratoconjunctivitis aims to control symptoms and prevent serious complications. The most important first step is avoiding allergens that trigger symptoms whenever possible^[10].

For mild cases, several simple treatments can provide relief. Cold compresses help reduce the urge to rub the eyes, which is important because rubbing can worsen the condition^[1][16]. Artificial tears, which are lubricating eye drops, help soothe the eye surface and wash away allergens and inflammatory substances^[10][16].

Medications form the main treatment approach. Antihistamines can be taken as eye drops or pills to relieve itching. These work by blocking the action of histamine, a chemical released during allergic reactions^[10][16]. Mast cell stabilizers are eye drops that prevent the release of inflammatory substances^[16].

For moderate cases, doctors may prescribe corticosteroids as eye drops. These are anti-inflammatory medications that reduce swelling and irritation. Low-potency topical corticosteroids can significantly improve symptoms when used as directed^[1][16]. In severe cases, oral corticosteroids may be necessary^[16].

For moderate to severe disease, immunosuppressants may be used. These include medications like tacrolimus or cyclosporine, which can be applied as ointments on the eyelids or taken by mouth. These drugs help control the overactive immune response without some of the side effects of long-term steroid use^[16].

In very severe cases where medical treatment is not enough, surgical procedures may be needed to address complications like cataracts or scarring of the cornea^[10]. However, it is important to note that there is currently no evidence from randomized controlled trials regarding the safety and effectiveness of systemic treatments specifically for atopic keratoconjunctivitis in children and young people^[7].

Living with the condition

Atopic keratoconjunctivitis is a chronic disease that requires long-term management. The condition follows a pattern of flare-ups and periods of improvement, with little connection to seasonal changes^[1]. Even people with mild cases need regular eye examinations to monitor for complications.

The disease can be quite debilitating for some people^[16]. Eye complications can occur quickly, particularly in younger patients with more severe disease. This makes it essential to follow treatment plans carefully and attend all scheduled appointments with eye care providers.

Effective management requires working with multiple healthcare specialists. People with this condition benefit from coordinated care involving dermatologists for skin problems, allergists for managing allergies, and ophthalmologists for eye care^[11]. Treatment can help control symptoms and prevent serious complications like scarring or ulcers in the eye^[2].

It is crucial not to stop medications without consulting a doctor, even if symptoms improve. In documented cases, people who discontinued their treatment experienced rapid worsening of their condition, leading to significant vision loss^[1]. With consistent treatment and monitoring, however, many people with atopic keratoconjunctivitis can maintain their vision and quality of life.