- Steven-Johnson syndrome (SJS) is a rare and serious condition of skin and mucous membrane which may be caused due to any illness or as side effect of any medication.It is named after Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in the American Journal of Diseases of Children in 1922. It is also known as Lyell’s syndrome.
- A more severe form of this is called toxic epidermal necrolysis (TEN) which involve more than 30 % skin surface and extensive damage to mucous membrane. SJS involves less than 10 % of skin.
- It is a medical emergency that usually requires hospitalization.
Signs and Symptoms of Steven-Johnson Syndrome
- SJS start with flu-like symptoms which include fever, sore throat, fatigue and burning eyes.
- As the condition develops, other signs and symptoms like skin pain, painful red or purple rash that spreads, blisters on skin and mucous membrane and shedding of skin after blister formation. Ulcer and other lesions mostly develop in mouth and lips (which make it difficult to eat and drink), but also in genital areas and anal region.
- Conjunctivitis occurs in about 30 % of children.
Causes of Steven-Johnson Syndrome
- It is mostly caused due to medication. If it is due to drug, symptoms tend to start about one to three weeks after the person has begun taking a medication. More than 100 drugs can cause SJS. Some common drugs are:
- Anti-gout medicines like allopurinol
- Anti-pain medicines like ibuprofen, acetaminophen and naproxen.
- Sulfonamide and sulfonamide like other medicines (carbonic anhydrase inhibitors).
- Anti-seizure medicine like carbamazepine, phenytoin and lamotrigine.
- Medicine used to treat mental illness.
- Antibiotics like azithromycin, vancomycin.
- It may be caused due to infection like mycoplasma pneumonia, otitis media, upper respiratory AIDs, influenza, cytomegalovirus and mumps. Use of antibiotics in bacterial infection makes difficult to identify the cause; either infection or medicine.
Diagnosis
- The diagnosis is based on involvement of less than 10 % of skin. A positive Nikolsyk’s sign is helpful.
Prevention
- Prevention can be done by screening individuals for certain variant genes before starting treatment with drugs that can cause SJS. For example, before starting treatment with carbamazepine, Taiwan and US FDA (Food and Drug Administration) recommend screening for HLA-B*15:02 in certain Asian groups.
- Any drug should be avoided if patient had already experienced SJS from that drug. It is very important to prevent recurrence which is more severe and fatal than first one.
Treatment
- The goal of treatment is to stop worsening of symptoms. In most of the cases, patients are hospitalized.
- If it is due to any medicine, the medicine is stopped immediately.
- Treatment involves supportive care. Various medicines may be used for various purpose. For example, eye drops to heal eye sore and prevent infection, antacids if sores grow in stomach and cause bleeding, antibiotics to treat infection in blood or applied locally to prevent infection, pain medicines to decrease pain and immune globulins to strengthen immune system.
- Electrolytes are replaced with IV fluids. Non-adhesive dressings are used for wound care.
- High calorie food is administered via tubing. TPN (liquid nutrition that provide our body with protein, vitamins, sugar, minerals and sometimes lipid) may be used.
- Surgery may be needed if skin doesn’t heal properly.
- An ophthalmologist should be consulted immediately as SJS may cause impaired vision, corneal vascularization and other ocular problems.