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  • Presentation

Treating the "Outsides" of the Inpatient: Pediatric Consults for the General Dermatologist; Severe Cutaneous Adverse Reactions

Description

Dr. Leah Layler presented a session focused on severe cutaneous adverse reactions in pediatric patients. She discussed two case studies involving severe skin reactions in young children. The first case centered on a three-month-old girl who developed a significant rash characterized by red papules and pustules after experiencing viral and bacterial infections. The diagnosis pointed towards acute generalized exanthematous pustulosis (AGEP), a reaction often triggered by infections rather than medications in younger children. The second case was about an eleven-year-old boy who presented with a blistering rash and systemic symptoms, ultimately diagnosed with a Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) overlap. He showed significant clinical deterioration despite treatment with TNF-alpha inhibitors, which led to a subsequent switch to cyclosporine that resulted in his gradual recovery. Dr. Layler emphasized the rarity and higher severity of cases in children, noting that idiopathic SJS is substantial among pediatric patients. She highlighted the challenges of identifying drug triggers and emphasized the need for prompt recognition and treatment of these severe reactions within the pediatric population.

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Conclusions

  • Acute generalized exanthematous pustulosis (AGEP) is rare in children and often has infectious triggers rather than medication triggers.
  • Diagnosis of AGEP can usually be made based on clinical presentation and biopsy findings.
  • DIRA and DITRA are rare genetic syndromes that can cause severe pustular reactions in neonates and require genetic testing for diagnosis and specific treatments.
  • Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are less common in children but can occur idiopathically or through drug triggers, often with no identified culprit in over 50% of cases.
  • Etanercept shows promise as a treatment for SJS/TEN, but may require earlier intervention for better outcomes.
  • Recurrent flares in children should prompt consideration of pustular psoriasis or genetic syndromes like DIRA/DITRA.
  • Current data suggests that while etanercept appears to lower mortality rates, its timing and dosing are critical for efficacy.
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