What is Stevens-Johnson Syndrome?

Stevens-Johnson Syndrome is a medical emergency that typically requires hospitalization. It is often a reaction to medications or infections and can start with flu-like symptoms before progressing to painful red or purplish rashes that spread and blister. Subsequently, the top layer of the affected skin dies, sheds, and then heals.

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Stages of Stevens-Johnson Syndrome

Initial Stage

The initial stage is marked by flu-like symptoms, including fever, sore throat, and fatigue.

Acute Stage

In the acute stage, skin and mucous membrane symptoms become apparent, including painful rashes and blisters. This stage requires immediate medical intervention.

Recovery Stage

During the recovery stage, the skin begins to heal, and systemic symptoms subside. Long-term care focuses on rehabilitation and managing any lasting complications.

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Causes of Stevens-Johnson Syndrome

Drug Reactions

One of the most common triggers for SJS is a reaction to medications. Drugs that are frequently implicated include:

Infections

Various infections can also precipitate SJS, including viral infections like herpes, hepatitis, and HIV, as well as bacterial infections.

Other Factors

Other risk factors that could contribute to the development of SJS include genetic predisposition and underlying autoimmune conditions.

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Symptoms of Stevens-Johnson Syndrome

Early Symptoms

The initial symptoms of Stevens-Johnson Syndrome often resemble the flu and may include:

Skin and Mucous Membrane Symptoms

Following the initial symptoms, individuals may experience:

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Diagnosing Stevens-Johnson Syndrome

Clinical Evaluation

Diagnosing SJS typically begins with a comprehensive medical history and physical examination. Physicians will look for characteristic signs and symptoms, particularly the pattern of skin and mucous membrane involvement.

Laboratory Tests

Laboratory tests may include blood tests to identify underlying infections or to rule out other conditions. Skin biopsies are often performed to confirm the diagnosis.

Imaging Studies

In some cases, imaging studies such as chest X-rays may be utilized to examine the extent of internal involvement, particularly if respiratory symptoms are present.

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Complications of Stevens-Johnson Syndrome

Acute Complications

Acute complications of SJS can be life-threatening and may involve:

Long-term Complications

Survivors of SJS may experience long-term complications, including:

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Treatment Options for Stevens-Johnson Syndrome

Hospitalization and Immediate Care

Due to the severity of SJS, hospitalization is almost always required. Immediate care focuses on:

Medications

Various medications may be administered to manage symptoms and prevent complications, including:

Wound Care

Wound care is an essential component of SJS treatment. This includes:

Long-term Care and Rehabilitation

Once the acute phase is managed, long-term care focuses on rehabilitation and managing chronic complications. This may involve:

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Risk Factors for Stevens-Johnson Syndrome

Genetic Factors

Certain genetic markers have been associated with an increased risk of SJS, particularly in response to specific drugs. For instance, the HLA-B*1502 allele is linked to a higher risk of SJS in individuals taking carbamazepine, especially among Asian populations.

Medical History

A history of SJS or a similar condition increases the likelihood of recurrence, particularly when re-exposed to the triggering drug.

Underlying Conditions

Individuals with HIV, lupus, or other autoimmune diseases are at a higher risk of developing SJS.

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Prognosis of Stevens-Johnson Syndrome

The prognosis of SJS varies depending on the severity and the timeliness of treatment. While some individuals recover fully, others may experience lasting complications. Mortality rates can be high, particularly in severe cases involving extensive skin detachment and multi-organ failure.

Factors Influencing Prognosis