Cornea is the outermost layer of our eye. It is clear and shaped like a dome. The cornea helps to shield the rest of the eye from germs, dust, and other harmful matter. It also helps your eye to focus. If you wear contact lenses, they float on top of your corneas.

Problems with the cornea include:

ALLERGIES

Steven Johnson’s syndrome

Physicians generally regard bold claims with an understandable mistrust. But every now and then a new treatment makes such a dramatic difference in patients’ life. That appears to be the case with the emerging use of amniotic membrane to alleviate the severe ocular manifestations of Stevens-Johnson syndrome (SJS).

Amniotic membrane has been used for some time in attempts to repair the damaged ocular surface after a bout of SJS. Only recently has it been applied during the onset of inflammation. The evidence thus far suggests that early treatment can mean the difference between preserving normal sight and surrendering to significant, permanent visual impairment.

Stevens-Johnson syndrome is a horrible disease the worst eye disease one can see. It’s like a burn in the eye. In the past, no one did anything about the eyes in the acute phase. They were struggling to save the patient’s life. But by the time you saved the patient’s life, they were blind.

Causes and Effects:

SJS is often an allergic reaction to certain common drugs, particularly sulfonamides, penicillins and non steroidal anti-inflammatories. Less frequently it can result from infections or malignancies.

The reaction presents with widespread exfoliation of the skin and mucous membranes, with clinical manifestations similar to those of partial – thickness burns. As a result, people who develop SJS are typically referred to burn units for treatment. In its most severe form toxic epidermal necrolysis the disease is frequently fatal.

Most SJS patients develop inflammation of the conjunctiva during the acute phase of the disease. When the inflammation is severe, it destroys the mucosa of the ocular surface and eyelids, leading to intractable dry eye, vision loss, severe chronic pain and photophobia.

SJS also affects the lid margins and eyelashes, distorting eyelash structure and increasing the risk of corneal abrasions.

If the corneal limbal stem cells are destroyed, the cornea can be covered by the surrounding conjunctiva and become vascularized, reducing the chances for a successful corneal transplant if needed in the future.

Stevens-Johnson syndrome presents a medical emergency that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications.

Recovery

Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. If your doctor determines that your case of Stevens-Johnson syndrome was caused by medication, you’ll need to permanently avoid the medication and all others related to it.

Signs and symptoms of Stevens-Johnson syndrome include:

• Facial swelling.
• Tongue swelling.
• Hives.
• Skin pain.
• A red or purple skin rash that spreads within hours to days.
• Blisters on your skin and mucous membranes, especially in your mouth, nose and eyes.
• Shedding (sloughing) of your skin.

If you have Stevens-Johnson syndrome, several days before the rash develops you may experience:

• Fever.
• Sore throat.
• Cough.
• Burning eyes.

Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience any of the following signs or symptoms:

• Unexplained widespread skin pain.
• Facial swelling.
• Blisters on your skin and mucous membranes
• Hives.
• Tongue swelling.
• A red or purple skin rash that spreads.
• Shedding of your skin.

Tests and Diagnosis

Doctors often can identify Stevens-Johnson syndrome based on your medical history, a physical exam and the disorder’s distinctive signs and symptoms. To confirm the diagnosis, your doctor may take a tissue sample of your skin (biopsy) for examination under a microscope.

Stevens-Johnson syndrome requires hospitalization, often in an intensive care unit or burn unit.

Stopping medication causes

The first and most important step in treating Stevens-Johnson syndrome is to discontinue any medications that may be causing it. Because it’s difficult to determine exactly which drug may be causing the problem, your doctor may recommend that you stop taking all nonessential medications.

Supportive care

Currently there are no standard recommendations for treating Stevens-Johnson syndrome.

Supportive care you’re likely to receive while hospitalized includes:

• Fluid replacement and nutrition. Because skin loss can result in significant loss of fluid from your body, replacing fluids is an important part of treatment. You may receive fluids and nutrients through a tube placed through your nose and advanced into your stomach (nasogastric tube).
• Wound care. Cool, wet compresses will help soothe blisters while they heal. Your health care team may gently remove any dead skin, and then place a dressing with a topical anesthetic over the affected areas, if needed.
• Eye care. Because of the risk of eye damage, your treatment should include consultation with an eye specialist (ophthalmologist).

Medications

Medications commonly used in the treatment of Stevens-Johnson syndrome include:

• Pain medication to reduce discomfort.
• Antihistamines to relieve itching.
• Antibiotics to control infection, when needed.

Topical steroids to reduce skin inflammation.

In addition, you may receive one of the following types of medications that are currently being studied in the treatment of Stevens-Johnson syndrome:

• Intravenous corticosteroids. For adults, these drugs may lessen the severity of symptoms and shorten recovery time if started within a day or two of when symptoms first appear. For children, they may increase risk of complications.
• Intravenous Immunoglobulin(IVIG). This medication contains antibodies that may help your immune system halt the process of Stevens-Johnson syndrome.

SKIN GRAFTING

If a large area of the body is affected, skin grafting removing skin from one area of the body and attaching it to another or using a synthetic skin substitute may be necessary to help heal. This treatment is only rarely required.

If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, the skin may begin to grow again within several days. In severe cases, full recovery may take several months.

LIFESTYLE AND HOME REMEDIES

If one has had Stevens-Johnson syndrome, be sure to:

• Know what caused the reaction. If the case of Stevens-Johnson syndrome was caused by a medication, learn the name of that medication and any other closely related medications that may cause the same reaction.
• Inform your health care providers. Tell the health care providers that one have a history of Stevens-Johnson syndrome. If the reaction was caused by medication, provide the caregivers with the name of that medication.

Wear a medical information bracelet or necklace. Have information about the condition and what caused it inscribed on a medical information bracelet or necklace, and wear it at all times.

PREVENTION

It’s difficult to prevent an initial attack of Stevens -Johnson syndrome because one does n’t know what trigger it. However, if Stevens-Johnson syndrome is got once, and the doctor determined that it was caused by medication, be sure to avoid that medication and others in the same class to prevent another attack. If the herpes virus caused the reaction, may need to take daily antiviral medications to prevent a recurrence.

A recurrence of Stevens-Johnson syndrome is usually more severe than the first episode and, in many cases, it can be fatal.

Authored By Dr. Neera Agrawal