Linear IgA Bullous Dermatosis in Adults and Children: A Narrative Review of Literature on Disease Distribution and Evaluation of Existing Scoring Tools.

Linear IgA Bullous Dermatosis (LABD) is a rare autoimmune blistering disease (AIBD) characterized by linear depositions of IGA antibodies at the dermo-epidermal junction, resulting in subepithelial vesiculobullous lesions that affect the skin and mucous membranes. Despite advancements in the study of AIBDs, significant gaps persist in LABD's clinical presentation, treatment, and outcome measures due to the absence of validated, disease-specific scoring systems. The purpose of this paper is to identify any differences in disease distribution between adults and children, and to review and evaluate existing scoring tool for AIBDs. A literature search was performed using Embase and PubMed to identify relevant studies and case reports. According to the literature, adults and children had different cutaneous distributions of disease, with lesions on the perineum, hands and feet, and face more frequently reported in children. However, there was inconsistent data on whether mucous membrane lesions were more frequent in adults or children. Current scoring systems for AIBDs were also identified, with several systemic scoring systems existing in the literature that are validated in scoring AIBD severity. There is currently no literature on scoring systems specific to LABD, underscoring the need to develop and validate LABD specific tools to facilitate the development of standardized treatment guidelines and advance the understanding and management of LABD.