Impaired secretory immunity in dystrophic epidermolysis bullosa

Dystrophic epidermolysis bullosa is a congenital disorder characterized by blistering of the skin and oral mucosa. This study investigated the hypothe sis that children with dystrophic epidermolysis bullosa have impaired oral secretory immunity. Immunoglobulin A (IgA), secretory IgA and IgG concentra tions, and IgA and secretory IgA antibody levels to Candida albicans, Lacto bacillus casei and Streptococcus mutans were measured in whole saliva from 22 children with dystrophic epidermolysis bullosa and 22 matched controls. Salivary total IgA and total IgG concentrations were significantly raised i n dystrophic epidermolysis bullosa due to serum leakage from oral blisterin g, but the converse was seen with secretory IgA. This suggestion of a mucos al immune defect was supported by decreased secretory IgA antibody response s to all three microorganisms tested. This apparent defect in secretory imm unity in dystrophic epidermolysis bullosa may be due to mucosal involvement and damage resulting in impaired antigen sampling in mucosal associated ly mphoid tissue or to impaired transport of secretory IgA across the salivary gland mucosa.