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