Stevens Johnson Syndrome

Stevens Johnson Syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It is usually a reaction to medication or an infection and requires immediate medical attention.

The condition often starts with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters. Eventually, the top layer of the affected skin dies, sheds, and then heals. Here, we explore the causes, symptoms, diagnosis, treatment, and prevention of Stevens-Johnson Syndrome.

Causes

The exact cause of Stevens-Johnson Syndrome is not always clear. However, it is often triggered by:

• Medications: Common culprits include antibiotics (especially sulfonamides), anti-seizure medications, and pain relievers such as ibuprofen and acetaminophen.
• Infections: Viral infections like herpes, pneumonia, HIV, and hepatitis are known triggers.
• Genetics: Some individuals may have a genetic predisposition making them more susceptible to SJS.

Symptoms

Symptoms of SJS can vary but typically include:

• Early symptoms: Fever, sore throat, and fatigue.
• Skin reactions: A painful red or purplish rash that spreads and blisters, particularly around the mouth, nose, eyes, and genitals.
• Mucous membrane involvement: Ulcers and lesions in the mouth, throat, eyes, and genital areas.

As the syndrome progresses, the affected skin may start to die and peel off, exposing raw, painful areas underneath. This can lead to severe complications such as dehydration, infection, and sepsis.

Diagnosis

Diagnosing Stevens-Johnson Syndrome typically involves:

• Medical history and physical examination: A healthcare provider will review the patient’s medication history and look for characteristic signs and symptoms.
• Skin biopsy: A sample of the affected skin may be examined under a microscope to confirm the diagnosis.
• Blood tests: These can help identify underlying infections or other possible triggers.

Treatment

Immediate medical treatment is crucial for individuals with SJS. Treatment may involve:

• Hospitalization: Often in an intensive care unit or burn unit for close monitoring and supportive care.
• Discontinuing the triggering medication: If a specific drug is identified as the cause, it will be immediately stopped.
• Supportive care: This includes pain management, fluid replacement, and wound care. Intravenous fluids and nutritional support may also be necessary.
• Medication: Corticosteroids and immune globulins may be used to reduce inflammation and suppress the immune system.
• Eye care: Ophthalmologists may need to treat eye involvement to prevent long-term damage.

Prognosis and Complications

The prognosis for individuals with Stevens-Johnson Syndrome can vary. While some people recover fully, others may experience long-term complications, including:

• Chronic skin problems: Scarring, pigmentation changes, and sensitivity to sunlight.
• Eye complications: Chronic dry eyes, vision loss, and severe conjunctivitis.
• Organ damage: In severe cases, SJS can affect internal organs leading to complications such as hepatitis or kidney failure.

Mortality rates for SJS can be high, particularly in severe cases, with estimates ranging from 5% to 25%.

Prevention

Preventing Stevens-Johnson Syndrome involves several strategies:

• Avoiding known triggers: If an individual has had a previous reaction to a medication, it is crucial to avoid that drug and related medications in the future.
• Genetic testing: In some cases, genetic testing may be recommended to identify individuals at higher risk, especially before starting certain medications.
• Medical alerts: Wearing a medical alert bracelet can inform healthcare providers of known drug allergies or past SJS episodes.

Nursing Care of Patients with Stevens Johnson Syndrome

Stevens-Johnson Syndrome (SJS) is a rare but serious disorder of the skin and mucous membranes. It is often a reaction to medication or an infection and requires immediate medical attention. The role of nursing in managing patients with SJS is crucial, as it involves comprehensive care that addresses both the acute and long-term needs of the patient.

Initial Assessment and Stabilization

The first step in managing a patient with SJS is a thorough initial assessment to determine the severity of the condition and identify any immediate threats to life:

• Vital Signs Monitoring: Constant monitoring of blood pressure, heart rate, respiratory rate, and temperature to detect any signs of hemodynamic instability or infections.
• Assessment of Skin and Mucous Membranes: Documenting the extent of skin detachment and mucous membrane involvement, noting areas of erythema, blistering, and necrosis.
• Fluid and Electrolyte Balance: Ensuring the patient is adequately hydrated, as fluid loss through damaged skin can be significant.

Acute Care Management

In the acute phase, nursing care focuses on stabilizing the patient, managing symptoms, and preventing complications:

Skin Care

Wound Care:

• Maintaining Skin Integrity: Using non-stick dressings, sterile techniques, and barrier creams to protect damaged skin and promote healing.
• Monitoring for Infection: Regularly assessing for signs of infection, such as increased redness, swelling, or purulent discharge, and ensuring prompt intervention.

Pain Management:

• Pharmacological Interventions: Administering prescribed analgesics and monitoring their effectiveness.
• Non-Pharmacological Interventions: Providing comfort measures such as cool compresses and positioning to alleviate pain.

Systemic Care

Fluid and Electrolyte Management:

• Intravenous Fluids: Administering fluids to maintain hydration and electrolyte balance.
• Monitoring Input and Output: Carefully tracking fluid intake and output to detect imbalances early.

Nutritional Support:

• Enteral Nutrition: Providing nutrition through a nasogastric tube if oral intake is insufficient or not possible due to mucosal involvement.
• Parenteral Nutrition: Administering nutrients intravenously if enteral feeding is contraindicated.

Infection Control

Preventing infections is a critical aspect of nursing care for patients with SJS:

• Isolation Practices: Implementing contact precautions to prevent the spread of infections.
• Strict Aseptic Technique: Using sterile techniques for wound care and invasive procedures.
• Antibiotic Stewardship: Administering prophylactic antibiotics as prescribed and monitoring for signs of infection.

Long-Term Management and Rehabilitation

After the acute phase, nursing care focuses on rehabilitation and supporting the patient’s recovery:

Physical Rehabilitation:

• Physical Therapy: Implementing exercises to maintain mobility and prevent contractures.
• Occupational Therapy: Assisting with activities of daily living and providing adaptive equipment as needed.

Psychosocial Support:

• Counseling Services: Offering psychological support to help patients cope with the emotional impact of SJS.
• Support Groups: Connecting patients with others who have experienced similar conditions to provide mutual support.

Patient Education and Preventive Measures

Educating patients and their families about SJS and its prevention is a key role of nursing care:

• Medication Management: Educating patients on the importance of avoiding known triggers and monitoring for early signs of recurrence.
• Genetic Testing: Informing patients about the option of genetic testing to identify potential risks before starting new medications.
• Medical Alerts: Advising patients to wear medical alert bracelets to inform healthcare providers of their condition.

REFERENCES

1. Kellerman RD, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis. In: Conn’s Current Therapy 2020. Elsevier; 2020. https://www.clinicalkey.com.
2. Tangamornsuksan W, et al. Relationship between the HLA-B*1502 allele and carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: A systematic review and meta-analysis. JAMA Dermatology. 2013;149:1025.
3. Darlenski R, et al. Systemic drug reactions with skin involvement: Stevens-Johnson syndrome, toxic epidermal necrolysis, and DRESS. Clinics in Dermatology. 2015; doi.org/10.1016/j.clindermatol.2015.05.005.
4. DermNet New Zealand. Stevens-Johnson syndrome/toxic epidermal necrolysis. https://www.dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis/.

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