Life Threatening Skin Rash series – Stevens Johnson Syndrome

Stevens Johnson Syndrome by EczemaBlues.com

Pictures taken from http://dermnetnz.org/reactions/sjs-ten.html

Stevens-Johnson Syndrome (SJS) is a rare, severe disorder that causes pain, red/purplish skin, blisters and shedding of skin. It can be drug-induced or due to infection. It is a medical emergency and takes from weeks to months to recover. It is more common in adults or older people and men who use more of the possible drugs that trigger the SJS.

What Causes Stevens-Johnson Syndrome?

Its cause is largely drug-induced (more than 70%) or due to infection. However, genetics, family history and weakened immune system are also risk factors.

From Mayo’s Clinic website:

  • Anti-gout medications, such as allopurinol
  • Pain relievers such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)
  • Medications to fight infection, such as penicillin
  • Medications to treat seizures or mental illness (anticonvulsants and antipsychotics)
  • Radiation therapy

The infectious causes listed by Medscape are:

Viral diseases:

  • Herpes simplex virus
  • AIDS
  • Coxsackie viral infections
  • Influenza
  • Hepatitis
  • Mumps

In children, Epstein-Barr virus, enteroviruses and upper respiratory tract infection.

Bacterial diseases:

  • Group A beta-hemolytic streptococci
  • Diphtheria
  • Brucellosis
  • Lymphogranuloma venereum
  • Mycobacteria
  • Mycoplasma pneumoniae
  • Rickettsial infections
  • Tularemia
  • Typhoid

 Symptoms of Stevens-Johnson Syndrome

  • Flu-like symptoms
  • Rash – Pain, Red, Purplish Skin
  • Blisters
  • Shedding Skin (Nikolsky’s sign)
  • Eye-related – Painful red eye, purulent conjunctivitis, photophobia, blepharitis

Diagnosis is usually made via physical examination or skin biopsy.

Stages of Stevens-Johnson Syndrome

  1. Fever, sore throat or mouth sores
  2. Fatigue, cough, headache
  3. Swelling (face, tongue), Hives
  4. Rash – Pain, Red, Purplish Skin, Symmetric on face and torso
  5. Formation of blisters on skin and mucous membranes of mouth, nose, eyes, genitals
  6. Shedding of skin

Types of Stevens-Johnson Syndrome

The types are categorized by the extent of the body surface area (BSA) affected:

  • Stevens-Johnson syndrome: Less than 10% BSA detached
  • Toxic epidermal necrolysis: More than 30% of the BSA detached

Treatment of Stevens-Johnson Syndrome

Finding out the cause, in particular for drug-induced cases, is critical. Discontinue the drug. Treatment is to reduce pain, control itch, prevent dehydration, infection and inflammation. Oral corticosteroids and antibiotics may be prescribed. The patient is usually hospitalized.

Similar to Pemphigus Vulgaris, mouth/blister care is required.

Care of the Skin

Care of the skin includes application of lotions and wet dressings (wound care, wet compress). Particular to Stevens-Johnson Syndrome, eye care is also important to clean and prevent dry eyes.

Complications of Stevens-Johnson Syndrome

Complications include sepsis (blood infection), secondary skin infection (cellulitis), eye inflammation (tearing and scaring of cornea and even blindness) and permanent skin discoloration and damage (including nails).

Anyone have had SJS? Do share in the comments, thanks loads for spreading encouragement and experience.

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