Dupilumab for Pediatric Linear Bullous Dermatosis: A Case Report and Literature Review.

INTRODUCTION: Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal blistering disease characterized by linear IgA deposition at the dermo-epidermal junction. While dapsone is the established first-line therapy, its use can be limited by the need for glucose-6-phosphate dehydrogenase screening and potential adverse effects. Novel treatments targeting the Th2 inflammatory pathway, such as dupilumab, are emerging as potential options.

CASE PRESENTATION: We present the case of a 17-year-old male with a 5-year history of pruritic vesicles and papules affecting 20% of his body surface area. The patient had failed multiple treatments, including high-potency topical steroids and oral prednisone tapers. Due to a preference to avoid dapsone and its associated monitoring, the patient was started on dupilumab (600 mg loading dose, followed by 300 mg every 2 weeks). Following initiation, the patient experienced a dramatic reduction in pruritus, with his itch scale improving from 9/10 to 1/10. Although active vesicle formation persisted, the patient reported high satisfaction and clinical improvement in lesion burden.

CONCLUSION: This case adds to the limited but growing body of evidence supporting the use of dupilumab in pediatric LABD. While it may not always result in lesion clearance as monotherapy, dupilumab's ability to significantly alleviate the debilitating pruritus associated with LABD makes it a compelling therapeutic alternative, particularly in refractory cases or when conventional systemic therapies are deferred. Further research is needed to determine the long-term efficacy and optimal positioning of dupilumab in the LABD treatment algorithm.