Stevens-Johnson syndrome (SJS) is a rare, severe skin reaction most often triggered by particular medications which affects skin and mucous membrane. It starts with flu-like symptoms, followed by the main symptom of Stevens-Johnson syndrome , formation of dusky-red painful patches, which lead to extensive skin blistering and peeling. The top layer of the skin, called the epidermis, detaches from the second layer of skin, called the dermis.
Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization. Treatment focuses on removing the cause, caring for wounds, controlling pain and minimizing complications as skin regrows. It can take weeks to months to recover.
If your condition was caused by a medication, you’ll need to permanently avoid that drug and others closely related to it.
Many cases of SJS happen in children and adults younger than 30 years old, but also occur in others, especially the elderly. More cases of SJS occur in females than males.
One to three days before a rash develops, you may show early signs of Stevens-Johnson syndrome, including:
• A sore mouth and throat
• Burning eyes
As the condition develops, other signs and symptoms include:
• Unexplained widespread skin pain
• A red or purplish rash that spreads
• Blisters on your skin and the mucous membranes of the mouth, nose, eyes and genitals
• Shedding of skin within days after blisters form
People with Stevens-Johnson syndrome will develop painful, raw areas of skin. The rash and blistering may involve other areas, such as the:
Causes of Stevens-Johnson syndrome include:
• Allergic reaction to a medication
• Infections, like mycoplasma pneumonia, herpes and hepatitis A.
• Graft-versus-host disease.
• No known cause.
If your case of SJS is caused by a drug, symptoms appear about one to three weeks after you start taking medication.
Drugs that can cause Stevens-Johnson syndrome include:
• Anti-gout medications, such as allopurinol
• Medications to treat seizures and mental illness (anticonvulsants and antipsychotics)
• Antibacterial sulfonamides (including sulfasalazine)
• Nevirapine (Viramune, Viramune XR)
• Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)
Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.
Risk Factors –
You are at greater risk of SJS if you have the following conditions:
• Bone marrow transplant.
• Systemic lupus erythematosus.
• Human immunodeficiency virus (HIV).
• Other chronic diseases of joints and connective tissue.
• Weakened immune system.
• Family history of SJS.
• Variation of a specific gene called human leukocyte antigen-B.
Stevens-Johnson syndrome complications include:
Areas where the skin has shed lose fluids. And sores in the mouth and throat can make fluid intake difficult, resulting in dehydration.
• Blood infection (sepsis).
Sepsis occurs when bacteria from an infection enter your bloodstream and spread throughout your body. Sepsis is a rapidly progressing, life-threatening condition that can cause shock and organ failure.
• Eye problems.
The rash caused by Stevens-Johnson syndrome can lead to eye inflammation, dry eye and light sensitivity. In severe cases, it can lead to visual impairment and, rarely, blindness.
• Lung involvement.
The condition may lead to acute respiratory failure.
• Permanent skin damage.
When your skin grows back following Stevens-Johnson syndrome, it may have abnormal bumps and coloring. And you may have scars. Lasting skin problems may cause your hair to fall out, and your fingernails and toenails may not grow normally.
Tests and procedures used to diagnose Stevens-Johnson syndrome include:
• A review of your medical history and a physical exam.
Doctors often can identify Stevens-Johnson syndrome based on your medical history, including a review of your current and recently stopped medications, and a physical exam.
• Skin biopsy.
To confirm the diagnosis, and rule out other possible causes, your doctor removes a sample of skin for laboratory testing (biopsy).
Skin or oral culture or culture from other areas may be taken to confirm or rule out infection.
Depending on your symptoms, your doctor may have you undergo an imaging such as a chest X-ray to check for pneumonia.
• Blood tests.
These are used to confirm infection or other possible causes.
People with Stevens-Johnson syndrome need to receive treatment in the hospital, often in an intensive care unit, burn unit, or dermatology unit.
Doctors will first want to identify whether a particular medication is causing Stevens-Johnson syndrome and discontinue its use as soon as possible. They may advise a person to stop taking all nonessential medications.
Doctors treat people with Stevens-Johnson syndrome with supportive care and medications.
Supportive care may include:
• extensive wound care
• pain management
• fluid and nutrition supplementation
• respiratory support
• eye care
• genital care
• keeping the room temperature between 86.0°F and 89.6°F
• monitoring the skin for infections
For wounds that appear in the mouth, doctors may recommend a disinfectant mouthwash. If the condition is affecting other areas, such as the eyes or genitalia, specialists will play a role in the person’s care.
Medications for treating Stevens-Johnson syndrome may include:
• pain relievers to lessen discomfort
• topical steroids to reduce inflammation
• antibiotics for infection control
For more informative articles on other health related issues, please visit our website www.santripty.com and also feel free to consult. Dr.Parveen and her team has risen up to the occasion to provide online video consultations and online follow-ups to help the needy patients during this pandemic situation.