Stevens Johnsons Syndrome

Stevens Johnsons Syndrome (SJS) is a rare vesiculobutbous disease and is characterized by an acute cutaneous eruption, which involves the skin and mucosal membranes. This condition was first of all described in 1922 as a generalized cutaneous eruption along with inflamed buccal mucosa and purulent conjunctivitis.

Causes of Stevens Johnsons Syndrome

The exact causes of SJS are not known. This disease is generally associated with drug therapy, infections agents and malignancy. Medication usage also leads to the development of Stevens Johnson syndrome.

Antibiotics are the medications that appear to have the strongest association with the disease along with sulphonamides and betalactam antibiotics. Other antibiotics, non-steroidal anti inflammatory agents and anticonvulsant drugs play a major role in the development of the disease.

Symptoms of Stevens Johnsons Syndrome

Generally, the Stevens Johnson syndrome, affects the mucous membranes of the oral cavity, nostril, eyes and both the anal and genital regions. It may or may not be associated with skin lesions anywhere else on the body.

Mouth lesions or Oropharyngeal lesions are sometimes so intolerable that they prevent eating and there may also be recurring oral ulcers. Commonly, the skin lesions may look like a target lesion or as bullae that is bubble like. There may be a painful conjunctivitis occurring in the eye.

This conjunctivitis may be usually associated with a pus discharge and it can lead to corneal scarring and loss of vision. Along with the occurrence of mucous membrane lesions, fever and exhaustion also commonly occur.

Near about one third of the patients have pulmonary involvement with cough and patchy infiltrates that is the material that has permeated into the tissues. It is commonly seen on chest X-ray. In fatal cases, renal failure and pneumonia may occur.

Because Stevens Johnsons Syndrome is a rare condition, hence estimates of its incidence and prevalence are challenging. The more severe disease forms, affect patients early or late in life. Stevens Johnson Syndrome is a member of a spectrum of inflammatory vesiculobullous diseases.

At the most benign end of the spectrum lies Erythema Multiforme Minor, which is characterized by an acute cutaneous eruption usually without mucosal membrane involvement. In Erythema Multiforme Major or Stevens Johnson Syndrome, the acute exanthem includes erosive involvement of two or more mucosal surfaces.

The most commonly involved surface is the oral mucosa. The conjunctiva is the next most commonly involved mucosal surface. Toxic Epidermal Necrolysis is the most severe vesiculobullous disease, which is characterized by mucosal and cutaneous lesions. SJS are particularly accompanied by systemic features such as fever, arthralgia, malaise and tenderness.

Treatment of Stevens Johnsons Syndrome

Every attempt should be made to identify a precipitating agent and to remove it if possible. Antibiotics are good if super infection is suspected, or if bacterial disease such as mycoplasma is thought to be the causative agent.

Intensive supportive care is necessary in case of severe cases, particularly specialized care in a burn unit. Fluid replacement is generally required and proper oral hygiene is necessary to prevent super infection. Precautions should be taken to avoid permanent eye damages. Oral and topical corticosteroids are often used.

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