Stevens Johnson Syndrome

Introduction:

Stevens-Johnson syndrome is a rare, serious and an immune-complex–mediated hypersensitivity complex disorder of skin and mucous membranes that usually appears as a reaction to some medication or an infection. In cases where it appears as a result of a reaction to some medication, patient requires to completely eliminate the use of that medication and also inform all his treating physicians about the same.

Specific characteristics of Stevens-Johnson syndrome

• Begins with flu-like symptoms
• Soon a painful red or purplish rash appears
• The rash spreads and forms blisters
• It affects the top layer of the skin
• Can also affect the body internally and makes eating, swallowing, even peeing difficult.

Causes of Stevens-Johnson syndrome

It is a rare and unpredictable syndrome in which it is difficult to identify the exact cause. But in most of the cases, it is usually triggered by a medication or an infection.

Drugs that can cause Stevens-Johnson syndrome include:

• Anti-gout medications, such as allopurinol
• Pain relievers such as acetaminophen, ibuprofen and naproxen sodium
• Medications to fight infection, such as penicillin
• Medications to treat seizures or mental illness
• Sulpha antibiotics that fights infections
• Radiation therapy

Infectious causes of Stevens-Johnson syndrome include

• Herpes (herpes simplex or herpes zoster)
• Pneumonia
• HIV or other immune system disorders
• Hepatitis

Risk factors of Stevens-Johnson syndrome

• Viral infections such as herpes, viral pneumonia, HIV or hepatitis.
• Weakened immune system due to organ transplant, HIV/AIDS and autoimmune diseases, such as lupus.
• A history of Stevens-Johnson syndrome
• A family history of Stevens-Johnson syndrome

Symptoms of Steven Johnson syndrome

Before the actual symptoms appear, patient may complain of following flu like symptoms:

• Fever
• Sore mouth
• Sore throat
• Chills
• Fatigue
• Headache
• Cough productive of a thick, purulent sputum
• Joint pain
• Burning sensations in the eyes
• Occasional vomiting or diarrhoea

Soon after the pre-symptomatic state, the real symptoms of the syndrome appear and patient complains of the following:

• Swelling on face and tongue
• Hives
• Skin pain
• A red or purple skin rash that looks burned
• Soon the rash gets peeled off and spreads within hours to days
• Blisters appear on skin and the mucous membranes of mouth, nose, eyes and genitals
• Shedding of skin

Specific eye related symptoms include:

• Eye irritation & itching
• Red eye
• Pain in eyes
• Heaviness in eyelids
• Excessive watery eyes
• Blepharospasm
• Burning sensations in the eyes
• Sand, gritty or foreign body feeling in the eye
• Corneal scarring
• Decreased or blurred vision
• Photophobia
• Blindness

Diagnosis of Stevens-Johnson syndrome

A thorough physical examination by the doctor along with skin biopsy is performed to diagnose Stevens-Johnson syndrome. Conjunctival biopsy an also be performed in patients who show clear ocular involvement with patient complaining of eye symptoms in his history.

Treatments of Stevens-Johnson syndrome

It is important to get the right treatment at right time to protect skin and other organs from lasting damage.

Stevens-Johnson syndrome is an emergency condition and requires hospitalization in an intensive care unit or burn unit. Treatment aims to control the symptoms, eliminate the cause and minimize complications including the following:

• Discontinue nonessential medications

• Wound care with cool and wet compresses to soothe
• Gentle removal of dead skin and medicated dressing over the affected areas
• Pain medication to reduce discomfort
• Antihistamines to relieve itching
• Antibiotics to control infection
• Topical steroids to reduce skin inflammation

Eye care: In cases where ocular manifestations are clear, aggressive eye treatment should be started to avoid any eye complication. It includes:

• Lubrication of the ocular surface
• Topical steroids, antibiotics, and symblepharon lysis
• Removal of keratinized plaques from the posterior lid margins
• Mucous membrane grafting
• Amniotic membrane grafting
• Adhesive glues
• Lamellar grafts
• Limbal stem cell transplantation
• Superficial keratectomy

Complications of Stevens-Johnson syndrome

If not caught on time or left untreated, the syndrome can spread drastically and affect whole body. It can lead to following complications:

• Cellulitis or secondary skin infection
• Sepsis or blood infection
• Inflammation of heart, lungs, kidney or liver
• Damaged skin even after the syndrome affected skin goes and fresh skin appears
• Eye complications like:
  • Dry eyes that hurt in bright light
  • Chronic cicatrizing conjunctivitis
  • Corneal epithelial defects
  • Corneal stromal ulcers
  • Corneal perforation
  • Endophthalmitis