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Emergency and Acute Medicine: Stevens–Johnson Syndrome
Stevens–Johnson syndrome (SJS) is a rare but severe mucocutaneous disorder characterized by widespread skin and mucosal injury. It involves blistering and epidermal detachment affecting less than 10% of the body surface area, distinguishing it from more extensive conditions such as toxic epidermal necrolysis. The disease almost always involves mucous membranes, typically affecting at least two sites such as the oral cavity, eyes, or genitalia. Skin lesions often begin on the face, neck, and trunk and may rapidly become confluent over hours to days. Although part of a spectrum with toxic epidermal necrolysis, SJS is considered a distinct clinical entity.
The condition is most commonly triggered by medications or infections. Drugs such as antibiotics (especially sulfonamides and penicillins), anticonvulsants, nonsteroidal anti-inflammatory drugs, and allopurinol are frequent culprits. Infectious causes include Mycoplasma pneumoniae and herpes simplex virus. The underlying mechanism involves an immune-mediated process in which cytotoxic T cells target keratinocytes expressing drug-related antigens, leading to widespread apoptosis of skin cells and systemic inflammation.
Patients typically present with a prodrome resembling a viral illness, occurring one to three days before the rash. Symptoms include fever, malaise, headache, upper respiratory symptoms, and sometimes joint or muscle pain. This is followed by skin tenderness, burning, and the development of erythematous or purpuric macules that may evolve into target-like lesions or flaccid blisters. These lesions can detach with slight pressure, demonstrating the Nikolsky sign. Painful mucosal involvement is a hallmark feature, leading to symptoms such as difficulty swallowing, urination discomfort, and eye irritation or conjunctivitis. Ocular involvement is common and may progress to serious complications such as corneal damage.
Diagnosis is primarily clinical and based on history, examination, and the extent of skin involvement. A careful medication history is crucial, as symptoms typically develop within two weeks of drug exposure, or more rapidly upon re-exposure. Laboratory tests may reveal nonspecific findings such as anemia or lymphopenia, while skin biopsy can confirm the diagnosis by showing full-thickness epidermal necrosis and subepidermal separation.
Management focuses on early recognition, withdrawal of the offending agent, and supportive care. Patients should be treated similarly to burn victims, especially those with significant skin involvement. Fluid resuscitation is essential due to fluid losses from denuded skin. Pain control is critical, as mucosal lesions can be extremely painful. Monitoring and treatment of secondary infections are vital, as sepsis—particularly from gram-negative organisms—is the leading cause of death. Advanced therapies such as intravenous immunoglobulin or corticosteroids may be considered, although their use remains controversial.
All patients with SJS require hospital admission, and those with extensive skin involvement should be managed in a burn unit or intensive care setting. Airway protection and ventilatory support may be necessary in severe cases. Long-term follow-up is important, particularly for ocular and dermatologic complications. Patients must be clearly educated about the causative drug and advised to avoid it permanently, as re-exposure can lead to rapid and more severe recurrence.
A key challenge is early recognition, as SJS often begins with nonspecific flu-like symptoms. Clinicians must maintain a high index of suspicion in patients presenting with mucocutaneous lesions and recent drug exposure. Prompt diagnosis and aggressive supportive care significantly improve outcomes.
Stevens–Johnson syndrome (SJS) is a rare but severe mucocutaneous disorder characterized by widespread skin and mucosal injury. It involves blistering and epidermal detachment affecting less than 10% of the body surface area, distinguishing it from more extensive conditions such as toxic epidermal necrolysis. The disease almost always involves mucous membranes, typically affecting at least two sites such as the oral cavity, eyes, or genitalia. Skin lesions often begin on the face, neck, and trunk and may rapidly become confluent over hours to days. Although part of a spectrum with toxic epidermal necrolysis, SJS is considered a distinct clinical entity.
The condition is most commonly triggered by medications or infections. Drugs such as antibiotics (especially sulfonamides and penicillins), anticonvulsants, nonsteroidal anti-inflammatory drugs, and allopurinol are frequent culprits. Infectious causes include Mycoplasma pneumoniae and herpes simplex virus. The underlying mechanism involves an immune-mediated process in which cytotoxic T cells target keratinocytes expressing drug-related antigens, leading to widespread apoptosis of skin cells and systemic inflammation.
Patients typically present with a prodrome resembling a viral illness, occurring one to three days before the rash. Symptoms include fever, malaise, headache, upper respiratory symptoms, and sometimes joint or muscle pain. This is followed by skin tenderness, burning, and the development of erythematous or purpuric macules that may evolve into target-like lesions or flaccid blisters. These lesions can detach with slight pressure, demonstrating the Nikolsky sign. Painful mucosal involvement is a hallmark feature, leading to symptoms such as difficulty swallowing, urination discomfort, and eye irritation or conjunctivitis. Ocular involvement is common and may progress to serious complications such as corneal damage.
Diagnosis is primarily clinical and based on history, examination, and the extent of skin involvement. A careful medication history is crucial, as symptoms typically develop within two weeks of drug exposure, or more rapidly upon re-exposure. Laboratory tests may reveal nonspecific findings such as anemia or lymphopenia, while skin biopsy can confirm the diagnosis by showing full-thickness epidermal necrosis and subepidermal separation.
Management focuses on early recognition, withdrawal of the offending agent, and supportive care. Patients should be treated similarly to burn victims, especially those with significant skin involvement. Fluid resuscitation is essential due to fluid losses from denuded skin. Pain control is critical, as mucosal lesions can be extremely painful. Monitoring and treatment of secondary infections are vital, as sepsis—particularly from gram-negative organisms—is the leading cause of death. Advanced therapies such as intravenous immunoglobulin or corticosteroids may be considered, although their use remains controversial.
All patients with SJS require hospital admission, and those with extensive skin involvement should be managed in a burn unit or intensive care setting. Airway protection and ventilatory support may be necessary in severe cases. Long-term follow-up is important, particularly for ocular and dermatologic complications. Patients must be clearly educated about the causative drug and advised to avoid it permanently, as re-exposure can lead to rapid and more severe recurrence.
A key challenge is early recognition, as SJS often begins with nonspecific flu-like symptoms. Clinicians must maintain a high index of suspicion in patients presenting with mucocutaneous lesions and recent drug exposure. Prompt diagnosis and aggressive supportive care significantly improve outcomes.
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