Eczema in primary immune-deficiencies. Clues to the pathogenesis of atopic dermatitis with special reference to the Wiskott-Aldrich syndrome.
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.
D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D003876:Dermatitis, Atopic / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D004485:Eczema / Q000209:etiology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D008297:Male; D014923:Wiskott-Aldrich Syndrome / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy