Updates in the Treatment of Pain and Pruritus in Epidermolysis Bullosa.

Pruritus and pain are pervasive, often debilitating symptoms in epidermolysis bullosa (EB), arising from complex immune, neural, and barrier dysfunction. Pruritus, especially in dystrophic and junctional EB subtypes, results from Th2/Th17-mediated inflammation, neuropeptide signaling, and neuropathic changes. Management includes antihistamines, topical agents, gabapentinoids, and emerging systemic therapies, such as dupilumab, JAK inhibitors, and neurokinin-1 inhibitors. Pain in EB is multifactorial, combining nociceptive and neuropathic mechanisms due to chronic wound, fibrosis, and nerve sensitization, requiring multimodal treatment strategies, including psychosocial therapies, analgesics, neuropathic agents, anti-inflammatory medications, and opioids. This review summarizes practical approaches to managing EB-associated pruritus and pain in the context of advancing insights into underlying pathophysiologic pathways.

Abstract from PubMed