Severe Stevens-Johnson Syndrome in an 11-Year-Old Male Complicated by Multi-Organ Failure

Case at a Glance

An 11-year-old male developed severe Stevens-Johnson Syndrome (SJS) following a course of amoxicillin-clavulanate. His condition rapidly progressed to involve significant skin desquamation, atypical pneumonia, and liver dysfunction, requiring intensive care, mechanical ventilation, and consideration for extracorporeal membrane oxygenation (ECMO).

Patient's Story

An 11-year-old male with a non-contributory past medical history was prescribed amoxicillin-clavulanate for a presumed infection. Several days into the antibiotic course, he developed a high fever and a painful, rapidly spreading rash. The rash evolved into blisters, and he developed severe erosions on his oral, ocular, and genital mucosal surfaces. Due to the rapid escalation of his symptoms, he was brought to a local hospital and promptly transferred to a tertiary care center with a pediatric intensive care unit (PICU) and a specialized burn unit.

Initial Assessment

Upon arrival at the PICU, the patient was febrile, tachycardic, and in acute respiratory distress. The physical examination revealed widespread erythematous macules, coalescing bullae, and epidermal detachment affecting approximately 25% of his total body surface area (TBSA). Severe, painful mucositis was noted. Initial laboratory studies showed leukopenia, elevated inflammatory markers, and significant transaminitis, indicating acute liver injury. After 10 days in the ICU, he remained sedated and on a ventilator.

The Diagnostic Journey

The diagnosis of Stevens-Johnson Syndrome was made based on the characteristic clinical presentation and the temporal relationship with the administration of amoxicillin-clavulanate. The offending drug was immediately discontinued. The patient's condition continued to deteriorate, necessitating intubation and mechanical ventilation for progressive respiratory failure. Chest radiography was consistent with atypical pneumonia, contributing to acute respiratory distress syndrome (ARDS). With worsening pulmonary and hepatic function, the critical care team began evaluating the patient for veno-venous ECMO as a potential bridge to recovery.

Final Diagnosis

Severe Stevens-Johnson Syndrome (SJS) secondary to amoxicillin-clavulanate, complicated by acute respiratory distress syndrome (ARDS), atypical pneumonia, and acute liver injury.

Treatment Plan

The patient was managed in the burn ICU with a multidisciplinary approach focused on aggressive supportive care:

• Supportive Care: Management was analogous to that for a severe burn patient, including meticulous wound care with non-adherent dressings, vigilant fluid and electrolyte resuscitation, thermoregulation, and nutritional support via a nasogastric tube.
• Organ Support: Continued mechanical ventilation with lung-protective strategies. The team remained on standby for ECMO cannulation if his respiratory status did not improve.
• Pharmacotherapy: All non-essential medications were ceased. Treatment for the secondary pneumonia was initiated with broad-spectrum antibiotics.
• Multidisciplinary Consultations:
  • Dermatology/Burn Surgery: Led daily wound care and monitored the progression of skin desquamation.
  • Ophthalmology: Provided aggressive ocular lubrication and daily assessments to prevent long-term complications like symblepharon and corneal scarring.
  • Speech-Language Pathology (SLP): A consultation was initiated to guide care. The SLP team recommended a strict oral hygiene protocol to reduce the risk of ventilator-associated pneumonia. They advised that a formal swallow evaluation (e.g., FEES or MBS) would be essential prior to reintroducing oral intake post-extubation, given the high risk of dysphagia from prolonged intubation and severe oropharyngeal mucositis.
  • Allergy/Immunology: Confirmed the likely causality and counseled the family on a lifelong allergy to penicillin and related beta-lactam antibiotics.

Outcome and Follow-up

The patient's prognosis remains guarded due to the severity of his condition and multi-organ involvement. While SJS/TEN carries significant morbidity and mortality, successful recoveries in pediatric patients have been well-documented, even in severe cases. The anticipated recovery will be prolonged, requiring extensive rehabilitation. Long-term follow-up will be crucial to manage potential sequelae, including skin scarring and pigmentary changes, chronic ocular disease, and potential pulmonary fibrosis. This case underscores the necessity of a highly coordinated, multidisciplinary team in managing the life-threatening complexities of SJS in the pediatric population.