Partial and Transient Clinical Response to Omalizumab in IL-21-Induced Low STAT3-Phosphorylation on Hyper-IgE Syndrome
oleh: Cesar Daniel Alonso-Bello, María del Carmen Jiménez-Martínez, María Eugenia Vargas-Camaño, Sagrario Hierro-Orozco, Mario Alberto Ynga-Durand, Laura Berrón-Ruiz, Julio César Alcántara-Montiel, Leopoldo Santos-Argumedo, Diana Andrea Herrera-Sánchez, Fernando Lozano-Patiño, María Isabel Castrejón-Vázquez
| Format: | Article |
|---|---|
| Diterbitkan: | Wiley 2019-01-01 |
Deskripsi
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 by Staphylococcus aureus. The dominant form is characterized by nonimmunologic features including skeletal, connective tissue, and pulmonary abnormalities in addition to recurrent infections and eczema. Omalizumab is a humanized recombinant monoclonal antibody against IgE. Several studies reported clinical improvement with omalizumab in patients with severe atopic eczema with high serum IgE level. We present the case of a 37-year-old male with HIES and cutaneous manifestations, treated with humanized recombinant monoclonal antibodies efalizumab and omalizumab. After therapy for 4 years, we observed diminished eczema and serum IgE levels.