Author/Authors :
Castrejón-Vázquez, Mar-a Isabel Immunology and Allergy Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Alonso-Bello, Cesar Daniel Immunology and Allergy Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Vargas-Camaño, Mar-a Eugenia Immunology and Allergy Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Lozano-Patiño, Fernando Immunology and Allergy Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Herrera-Sánchez, Diana Andrea Immunology and Allergy Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Jiménez-Mart-nez, Mar-a del Carmen Faculty of Medicine - Universidad Nacional Aut ́onoma de M ́exico, Mexico , Hierro-Orozco, Sagrario Dermatology Department - Centro M ́edico Nacional 20 de Noviembre - Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico , Ynga-Durand, Mario Alberto Universidad Nacional Aut ́onoma de M ́exico - Facultad de Estudios Superiores Zaragoza - Facultad de Medicina, Mexico , Alcántara-Montiel, Julio César Universidad Nacional Aut ́onoma de M ́exico - Facultad de Estudios Superiores Zaragoza - Facultad de Medicina, Mexico , Berrón-Ruiz, Laura Immunodeficiencies Research Unit - Instituto Nacional de Pediatria - Secretar ́ıa de Salud, Mexico , Santos-Argumedo, Leopoldo Department of Molecular Biomedicine - CINVESTAV-IPN, Mexico
Abstract :
Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by elevated levels of immunoglobulin E (IgE),eczematous dermatitis, cold abscesses, and recurrent infections of the lung and skin caused byStaphylococcus aureus.Thedominantform is characterized by nonimmunologic features including skeletal, connective tissue, and pulmonary abnormalities in additionto recurrent infections and eczema. Omalizumab is a humanized recombinant monoclonal antibody against IgE. Several studiesreported clinical improvement with omalizumab in patients with severe atopic eczema with high serum IgE level. We present thecase of a 37-year-old male with HIES and cutaneous manifestations, treated with humanized recombinant monoclonal antibodiesefalizumab and omalizumab. After therapy for 4 years, we observed diminished eczema and serum IgE levels
Keywords :
Partial , Transient Clinical Response , Omalizumab in IL-21-Induced , Low STAT3-Phosphorylation , Hyper-IgE Syndrome , Hyper-IgE syndrome (HIES)
