Congenital immunodeficiency syndromes with cutaneous manifestations. II

This article reviews different types of skin conditions (cutaneous manifestations) associated with congenital (present at birth) immunodeficiency syndromes. Immunodeficiency syndromes are diseases that suppress the immune system, thereby increasing the risk of opportunistic infection. Mucocutaneous candidiasis is caused by a microorganism called Candida. It can cause infections of the scalp, mouth and esophagus, and can cause abnormalities in the gonads and thyroid. It is treated with ketoconazole or amphotericin B antibiotics. Hyperimmunoglobulin E syndrome (overproduction of immunoglobulin type E) begins in the first three months of life. It is associated with skin inflammation (dermatitis), burning, itching and scaling, and with pneumonia. Staphylococcus and streptococcus are believed to be the causative agents, and the dermatitis is treated with antibiotics. Immunoglobulin A (IgA) deficiency is the most common inherited immunodeficiency syndrome. It occurs in 1 out of 400 to 600 people. It is associated with arthritis, anemia, respiratory tract infection, inflammation of the ear (otitis media), allergic disorders, chromosome abnormalities, and liver disease. Severe combined immunodeficiency syndrome involves several different disorders and occurs within the first three months of life. It is associated with skin and lung infections, and diarrhea caused by viruses. It is treated by bone marrow transplantation. Wiskott-Aldrich syndrome and agammaglobulinemia (lack of cells that produce antibodies) are diseases linked to the X chromosome. These syndromes are associated with infection of the skin, ears, eyes and brain membranes. Wiskott-Aldrich syndrome is treated by bone marrow transplantation, and agammaglobulinemia with gamma-globulin and antibiotics. (Consumer Summary produced by Reliance Medical Information, Inc.)