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Stevens Johnson Syndrome

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What is Stevens-Johnson Syndrome?

Stevens-Johnson syndrome (SJS) is a rare skin and mucous membrane disorder. It occurs in response to medication most often but can also occur in response to infections. In children, infections are the most common cause. In adults, medications are the most common cause. It happens more often in females than males. Most patients are between the ages of 10-30 years old but can occur in children even a few months old. The risk of contracting SJS increases with cases of immunosuppression such as cancer or HIV. There is a genetic component at play so family history can predispose you to SJS. The condition results in skin blistering, rashes, and necrosis. The condition presents similar to that of extensive burns and the patients are very ill. SJS patients are usually unable to function normally, daily tasks such as eating are very difficult. These patients are at higher risk of infection or death. Stevens-Johnson can have negative effects on the eye because of its tendency to attack mucous membranes. Limbal stem cell failure is common, decreasing the cornea’s ability to regenerate and heal. Therefore, corneal damage and scarring can occur. Scarring of the front surface of the eye can lead to symblepharon, adhesion between the eyelids to the eyeball. This can lead to decreased tear flow and disruption of the eye surface, leading to severely dry eyes. Vision loss can sequentially occur from accruing damage. Stevens Johnsons syndrome can result in many negative effects on the eyes that may decrease the patient’s quality of vision and everyday living. Your eye doctor can work with you to prevent detrimental effects and initiate proper treatment in order to improve your visual prognosis. Luckily, there are many treatment options available that your eye doctor can use that would improve comfort and vision.


Risk factors/Causes

Risk factors for Stevens Johnson's syndrome ranges from genetics to immune status to environmental causes. You are at a greater risk of developing this condition if you have any type of immune suppression such as bone marrow transplant, lupus, or HIV. Cancer or chronic diseases of connective tissue can also be potential causes. A family history or a personal history of SJS can predispose you to the condition. Infections can also be associated with the development of SJS. Some examples of infections that have been linked to the condition include: Herpes, Hepatitis, walking pneumonia, histoplasmosis, and adenovirus.


Signs and Symptoms

Due to the effects on mucous membranes, some common symptoms of SJS include:

  • Blisters and sores on mouth, throat, genital areas, and eyes
  • Peeling skin
  • Drooling
  • Eyelids that blister and swell, resulting in sealed lids
  • Painful urination
  • Cough
  • Body ache
  • Fever
  • Skin rashes
  • Lesions on the skin that have the appearance of a target

Complications

Stevens Johnsons can become a fatal condition, the most serious complication being death. However, this only happens in a select number of cases. Other serious complications of the condition involve pneumonia, bacterial blood infections that can lead to sepsis, shock, or multiple organ failure. Some long-term complications involve hair loss, lung damage, chronic fatigue, loss of taste, dry skin, light sensitivity, and chronic swelling or irritation of the eyes. In more serious cases, it may lead to vision impairment or even blindness.

A list of some eye conditions that may occur due to Stevens-Johnson syndrome include:

  • Iritis/ Conjunctivitis—inflammation in the eye
  • Corneal ulcers and erosions
  • Corneal holes
  • Blistering of the front surface of the eye
  • Conjunctival scarring
  • Permanent vision loss
  • Dry eye
  • Meibomian gland dysfunction
  • Limbal stem cell failure
  • Neovascularization of the cornea
  • Symblepharon or ankyloblepharon—sticking of the lids to the eye surface or lids to lids

Diagnosis

The diagnosis of Stevens-Johnson syndrome is primarily done by a dermatologist. They will make a diagnosis from a combination of symptoms, thorough medical history, and a physical examination. A skin biopsy can be done to confirm the diagnosis. In terms of eye related complications of SJS, this is done primarily by an ophthalmologist or optometrist. Your eye doctor can help diagnose corneal ulcers and infections, dry eye, and other ocular conditions. Your eye doctor will be able to initiate proper treatment and help improve comfort and vision.


Treatment

Some examples of treatment options for eye related complications of SJS include:

  • Topical corticosteroids and antibiotics
  • Bandage contact lenses
  • Scleral lenses
    • Scleral contact lenses are one of the most widely used treatments for eyes afflicted with SJS. Scleral lenses provide an immense benefit to these patients. A scleral lens has a large diameter that sits on the white part of your eye instead of the cornea. This allows for a fluid reservoir to sit between the cornea and the lens, lubricating and hydrating the front surface of the eye. It also serves as a protective barrier to prevent additional damage from the mechanical friction associated with blinks. The lenses will help prevent scarring and promote overall healing. Scleral lenses can better improve vision from scars that have occurred over that of a soft contact lens.
  • Corneal transplants
  • Artificial corneal procedures
    • Corneal transplants are typically not successful in patients with SJS. Therefore, an artificial cornea can be used as an alternative procedure.
  • Amniotic membrane
    • The use of amniotic membranes enhances the ability of the eye to heal, reduces neovascularization, and prevents eye surface scarring. The amniotic membrane is comprised of a plethora of immune cells and growth factors that assist in eye healing.

Some general treatment options for SJS involve:

  • IV fluids
  • Wound care
  • Steroids
  • Pain medications
  • Antibiotics

Prevention

In some cases, it is hard to prevent SJS because often we do not know what specific reactions we have to medications until it happens. If you are already aware of your reaction to certain medications, the key to prevention would simply be avoiding that drug. If a family member or yourself has experienced SJS from a medication, then you should all work to avoid that medication as there could be a genetic component to the reaction. Genetic testing can help determine whether you carry the genes associated with an increased risk of contracting the syndrome.

In order to prevent ocular complications, it is imperative to treat stevens-johnson syndrome as soon as possible. Amniotic membranes can be applied to the eyes or lids in early stages of the condition in order to prevent severe visual problems. This can help prevent any scarring that characterizes the advanced stages of the syndrome. The use of scleral contact lenses can also serve to protect the eye from mechanical irritation caused by blinking. It can also serve to lubricate the eye and prevent irritation from dryness. These treatments can help with symptoms and improve overall patient comfort while kickstarting the healing process.


FREQUENTLY ASKED QUESTIONS ABOUT SJS

Is there a different name for Stevens Johnsons?

SJS is also referred to as Lyell’s syndrome and toxic epidermal necrolysis.

What drugs are most commonly associated with the development of SJS?

Stevens Johnsons is typically associated with the use of sulfa based antibacterial drugs. Some less commonly known causes of SJS would be anti-seizure medications such as phenytoin, gout medications such as allopurinol, non-steroidal anti-inflammtoru drugs such as piroxicam, and other antibiotic classes.

Is there anything I can do to prevent Stevens Johnson's?

Steven Johnson's is triggered by factors that are typically out of our control. There is no way to know if you have a serious adverse reaction to a drug before you take it which is the most common cause of SJS. If you know you have a drug allergy, it is imperative to avoid this drug in the future.

If I get SJS once can I get it again?

Yes, SJS has the potential to redevelop if you one were to be re-exposed to the offending pathogen or medication. The second episode is typically more severe than the first and may even result in death. Therefore, it is recommended to avoid the original cause of the condition if possible.

Would my family members be at risk at SJS if I am prone to developing it?

There is a genetic component to the condition. Therefore, your family members may also be at risk of developing SJS. They should avoid the drug that had caused SJS for you.

What is the general prognosis for a Stevens-Johnson patient?

Most patients have long term complications from SJS, involving the skin or eyes. Some eye related complications that may result involve light sensitivity, dry eyes, synechiae, meibomian gland dysfunction, or vision loss.

Will my dry eye go away once I am treated?

SJS results in irreversible damage to the eye surface, glands, and eyelids. This prevents normal functioning of the glands that secrete tears, causing chronic dryness. Your eye doctor will be able to help you improve your daily living by decreasing feelings of grittiness and dryness.

How long does it take for Stevens-Johnson syndrome to develop?

The condition typically begins within a month following exposure to the medication or other causative agent. Very rarely does the condition develop within hours.