European S2k guidelines on management of autoimmune blistering diseases in children and adolescents

Introduction: Autoimmune blistering diseases (AIBDs) in children and adolescents present a major challenge in diagnosis and management because of their rarity and the presence of other differential diagnoses with blistering. They are potentially serious, and timely diagnosis and optimal management can help prevent morbidity related to both the diseases and their treatments. Methods: The Autoimmune Blistering Diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated evidence-based, expert-informed clinical guidelines for the diagnosis, treatment and monitoring of paediatric AIBDs. A writing group conducted a systematic review of the available literature on AIBDs in children and adolescents, and since there is limited strong evidence in the literature, recommendations were formulated by consensus over three rounds of structured feedback and revision by the Core Group and Expert Group of the task force (32 voting experts; >75% agreement threshold). Results: Although all AIBDs—including intra-epidermal and subepidermal blistering disorders—are reported in children, linear IgA dermatosis (LAD) is the most common (42–83%). The diagnostic approach in the paediatric age group does not differ from that in adults. Treatment recommendations are tailored to paediatric patients, taking into account developmental pharmacokinetics, potential adverse effects and the impact of disease and therapy on quality of life. Conclusions: These guidelines provide the first comprehensive, paediatric-focused framework for the diagnosis and management of AIBDs. Implementing the recommendations should harmonize care, reduce treatment-related toxicity and improve quality of life for affected children while steering future research towards critical knowledge gaps.