Salivary function of persons with hereditary epidermolysis bullosa

Oral alterations of the hard and/or soft tissues are commonly associated with the different types of epidermolysis bullosa (EB). The relationship of oral soft and hard tissue changes to the disease mechanisms in different EB types remains to be elucidated. The purpose of this investigation was to evaluate selected aspects of salivary function in a healthy control population and in persons affected with different types of EB. Sixty-one patients with EB, representing all the major types of EB, and 36 unaffected persons were examined to measure their stimulated salivary flow rates and salivary levels of IgA, albumin, and total protein. Our results show that none of the types of EB demonstrated a decreased salivary flow rate. However, patients with recessive dystrophic EB had significantly elevated salivary IgA, albumin, and total protein levels. The increased IgA level seen in this form of EB appears most likely to be related to the high prevalence of oral blistering rather than the result of altered mucosal immune function. Despite severe cutaneous and extracutaneous involvement associated with inherited EB, we found no evidence to support the hypothesis of abnormal salivary function or mucosal immunity in this disease. Taken together, these findings suggest that the rampant dental caries seen in the severe forms of EB are likely attributable to nonsalivary factors such as enamel involvement, soft tissue alterations, and/or diet. Alternatively, there may be mucosal immunity or salivary enzyme alterations that influence oral disease in these patients, but these were not evaluated in this investigation.