Upper aerodigestive tract complications in a neonate with linear IgA bullous dermatosis

Abstract

Linear IgA bullous dermatosis (LABD) is a rare immunobullous condition known to affect the skin and mucous membranes of the eye and oral cavity in adults and young children. We describe a newborn with skin involvement who had life-threatening respiratory compromise from disease affecting the larynx, subglottis, trachea, and esophagus. Management with both tracheostomy and gastrostomy tube placement was necessary. Treatment included systemic steroids, dapsone, and intravenous immunoglobulin. We compare our neonatal case to the only other report in the literature highlighting involvement of the mucous membranes of multiple levels of the aerodigestive tract leading to respiratory compromise.