Eczema in primary immune-deficiencies. Clues to the pathogenesis of atopic dermatitis with special reference to the Wiskott-Aldrich syndrome

Abstract

Eczema is one of the cutaneous manifestations of primary immune deficiencies. It may therefore serve as a model for the understanding of atopic dermatitis (AD) provided the eczema in the immune deficiency under consideration is a constant feature and is similar to AD. In the Wiskott-Aldrich syndrome the eczematous eruption is (i) a constant feature of the syndrome, (ii) indistinguishable from AD when diagnostic criteria for AD are used, and (iii) clears dramatically after a successful bone marrow graft, which suggests that immune dysfunction is directly involved in the pathogenesis of the eczema.