The increased prevalence of allergic disease in anhidrotic congenital ectodermal dysplasia

Abstract

Anhidrotic congenital ectodermal dysplasia (ACED) is a sex-linked recessive disorder which is characterized by anodontia or severe hypodontia, anhidrosis or severe hypohidrosis, and hypotrichosis. Afflicted individuals may also demonstrate evidence of hypoplastic changes in the respiratory mucosa. Seven male patients with ACED were found to have a statistically significant increased prevalence of extrinsic bronchial asthma and/or allergic rhinitis and an increased prevalence of positive prick test reactions to common inhalant allergens when compared with a control population examined in the Tecumseh Community Health Study. Four patients with ACED and allergic disease had no consistent abnormality in serum IgG, IgA, or IgM levels, and both transport piece and IgA were demonstrable in their salivary secretions or parotid fluid. It is postulated that the increased prevalence of allergic disease in patients with ACED may be due to an abnormality of the nasal mucosa resulting in an increased permeability to or decreased destruction of inhaled allergens.