Autosomal Dominant Severe Epidermolysis Bullosa Simplex Treated With Dupilumab.

Epidermolysis bullosa simplex (EBS) is an inherited mechanobullous disorder with limited systemic treatment options. Emerging evidence suggests that inflammatory pathways and pruritus may significantly contribute to disease severity, creating the possibility for therapeutic targets. This report describes a premature infant with EBS, severe (EBS-sev) and failure to thrive, who was treated with dupilumab at 5 months and 12 days of age. Treatment resulted in sustained improvement in skin integrity and clinical stability, suggesting that dupilumab may be considered as a potential therapeutic option for future cases of EBS.

Abstract from PubMed