Atopieassoziierte syndrome

In this study of the literature the association between atopic dermatitis (AD) and other hereditary conditions is reviewed. The most important observations are related to the primary immunodeficiency syndromes, such as Wiskott-Aldrich syndrome, selective IgA deficiency, and hyper-IgE syndrome. These are interesting from the pathogenetic point of view with respect to the immunologic mechanisms leading to AD. In contrast, the significant association of AD with the ectodermal dysplasias (ED), e.g. anhidrotic ED Christ-Touraine-Siemens and other ED, i.e. associated with clefting and limb abnormalities, is more problematic to interpret, in as much as little is known about the genetic background of this association. Finally the associations with metabolic diseases (e.g. phenylketonuria), ichthyosis and the syndromes of Down, Dubowitz and Konigsmark-Berlin-Hollander are discussed.