ABSTRACT
PID syndromes are a heterogeneous group of diseases characterized by:
• Increased susceptibility to infections • Autoimmunity and allergic disorders • Cancer proneness • Prominent cutaneous signs
For the purpose of this chapter, we will consider only the major forms with relevant skin signs and some rarest disorders with peculiar traits:
• Ataxia-telangiectasia • Chediak-Higashi syndrome • Cartilage-hair hypoplasia • Chronic granulomatous disease • Chronic mucocutaneous candidiasis • Autoimmune polyendocrinopathy-candidiasis-
ectodermal (enamel) dysplasia (APECED) syndrome
• Hyper-immunoglobulin E (IgE) syndromes • Hereditary angioedema (HAE) • Omenn syndrome-severe combined immunode-
ficiency • Common variable immunodeficiency • Wiskott-Aldrich syndrome • Immunoglobulin deficiencies • Cyclic neutropenia • Leukocyte adhesion deficiencies • DiGeorge’s syndrome • Fanconi anemia
Louis-Bar syndrome.
