Anhidrotic ectodermal dysplasia with frequent infections and amyloidosis

A 44 year-old male patient is described with anhidrotic ectodermal dysplasia, frequent cutaneous and respiratory infections and amyloidosis with nephrotic syndrome. The increased susceptibility to infections appeared to be related primarily to local factors since phagocytic and immune function were intact. Amyloidosis, a previously unreported complication of anhidrotic ectodermal dysplasia, probably developed as a consequence of the recurrent infections.