Overview
Definition:
Stevens-Johnson syndrome (SJS) is a rare, severe, and potentially fatal mucocutaneous reaction, typically triggered by medications
It is characterized by widespread epidermal necrosis and mucous membrane involvement, often accompanied by systemic symptoms
SJS is considered part of a spectrum with toxic epidermal necrolysis (TEN), with SJS defined by <10% body surface area detachment and SJS-TEN overlap by 10-30% detachment.
Epidemiology:
The incidence of SJS is estimated to be 1-6 cases per million person-years
Certain medications, particularly allopurinol, sulfonamide antibiotics, antiepileptics (e.g., carbamazepine, lamotrigine, phenytoin), and non-steroidal anti-inflammatory drugs (NSAIDs), are common culprits
Genetic predisposition, such as HLA-B*1502 allele in Southeast Asian populations for carbamazepine, plays a role.
Clinical Significance:
SJS is a medical emergency requiring prompt recognition and management
Mortality rates can be significant (5-15%), primarily due to complications like sepsis, dehydration, electrolyte imbalance, and organ failure
Understanding admission criteria and providing robust supportive care are paramount for improving patient outcomes in pediatric patients.
Admission Criteria
Severity Assessment:
Admission to an intensive care unit (ICU) or a specialized burn unit is indicated for patients with SJS/TEN
Criteria include extensive mucous membrane involvement (oral, ocular, genital), >10% body surface area epidermal detachment, significant systemic symptoms (e.g., fever >38.5°C, hypotension, respiratory distress), rapidly progressive rash, or evidence of organ involvement (e.g., hepatitis, nephritis, pneumonia).
Pain Management Needs:
Severe pain due to widespread skin lesions and mucous membrane erosions necessitates aggressive pain control, often requiring parenteral analgesics
Adequate pain management improves patient comfort and can facilitate essential nursing care and mobilization.
Fluid And Electrolyte Balance:
Significant fluid losses from denuded skin can lead to hypovolemia and electrolyte disturbances
Patients requiring intravenous fluid resuscitation, close monitoring of intake and output, and electrolyte correction warrant hospital admission, often in a high-dependency setting.
Nutritional Support Requirements:
Oral intake is often impossible due to painful mucositis
Patients requiring nasogastric (NG) tube feeding or parenteral nutrition for adequate caloric and protein intake need hospital admission and monitoring for nutritional status and complications.
Supportive Care
Skin Care:
Gentle wound care is crucial to prevent secondary infection and promote healing
This includes debridement of loose epidermis, topical antiseptic application (e.g., chlorhexidine), and non-adherent dressings
Avoidance of occlusive dressings unless specifically indicated helps with evaporative fluid loss monitoring.
Mucous Membrane Care:
Aggressive oral and ocular care is essential
Frequent saline or antiseptic mouthwashes, topical anesthetics (e.g., lidocaine gel), and diligent lubrication of eyes with artificial tears or ointments can alleviate pain and prevent complications like corneal ulceration and synechiae
Consult ophthalmology early.
Fluid And Electrolyte Management:
Intravenous fluid replacement is critical to maintain hemodynamic stability and adequate urine output
Isotonic crystalloids are typically used, with careful monitoring of serum electrolytes, renal function, and fluid balance
Management of hypothermia is also important due to skin barrier loss.
Nutritional Support:
Enteral feeding via NG or nasojejunal tubes is preferred if possible to maintain gut integrity
High-calorie, high-protein formulations are recommended
If enteral feeding is not feasible, parenteral nutrition may be necessary
Nutritional support should be initiated early.
Pain Management:
A multimodal approach is usually required, combining intravenous opioids (e.g., morphine, fentanyl) with non-opioid analgesics
Patient-controlled analgesia (PCA) can be effective
Management of neuropathic pain may also be needed
Regular reassessment of pain scores is vital.
Pharmacological Management
Drug Withdrawal:
Immediate and permanent discontinuation of the suspected offending drug is the cornerstone of management
Identifying the culprit drug is paramount, and a detailed drug history is essential.
Supportive Medications:
Antibiotics are used judiciously to treat documented secondary bacterial infections
Antipyretics for fever
Opioid analgesics for pain
Topical corticosteroids may be used cautiously on early lesions but are generally avoided on denuded areas due to infection risk.
Immunomodulatory Therapies:
The role of systemic corticosteroids is controversial and not definitively proven to improve outcomes
they may increase the risk of infection
Intravenous immunoglobulin (IVIG) has shown some promise in certain studies, potentially by blocking Fas-mediated keratinocyte apoptosis, but its efficacy remains debated
Cyclosporine has also been used with some success
Decisions regarding immunomodulatory therapy should be individualized and made in consultation with experts.
Monitoring And Complications
Vital Sign Monitoring:
Close monitoring of vital signs, including temperature, heart rate, respiratory rate, blood pressure, and oxygen saturation, is essential to detect early signs of sepsis, hypovolemia, or respiratory compromise.
Infection Surveillance:
Patients are at high risk for sepsis due to the compromised skin barrier
Vigilant monitoring for signs of infection (e.g., fever, leukocytosis, purulent drainage) and prompt empirical antibiotic therapy if suspected is crucial
Blood, urine, and wound cultures should be obtained.
Ophthalmic And Mucosal Surveillance:
Regular ophthalmological and otorhinolaryngological assessments are necessary to detect and manage complications such as conjunctivitis, corneal ulceration, iritis, urethritis, and severe anal or vaginal involvement
Early intervention can prevent long-term sequelae.
Renal And Hepatic Function:
Monitoring of renal and hepatic function tests is important, as these organs can be affected by the drug reaction or secondary complications
Adequate hydration is key for preventing acute kidney injury.
Key Points
Exam Focus:
Prioritize recognition of SJS/TEN spectrum, immediate drug withdrawal, and aggressive supportive care
Understand the SCORTEN score for prognostication
Differentiate SJS from less severe drug eruptions.
Clinical Pearls:
Always obtain a thorough drug history, including over-the-counter medications and traditional remedies
Early consultation with Dermatology, Ophthalmology, and Critical Care is vital
Pain management and fluid resuscitation are critical initial steps.
Common Mistakes:
Delaying withdrawal of the offending agent
Underestimating the severity and fluid requirements
Inadequate pain control
Overuse or delayed use of antibiotics
Misdiagnosing SJS as a simple viral exanthem or urticarial rash.