Role of omalizumab in a patient with hyper-IgE syndrome and review dermatologic manifestations.
Hyper-IgE syndrome (HIES) is a rare idiopathic primary immunodeficiency. It is characterized by a triad of findings, including high levels of serum IgE, recurrent skin abscesses and pneumonia and leads to pneumatocele formation. The diagnosis of HIES is complicated by a diversity of clinical and immunological spectrums and a heterogeneous set of genetic defects. The National Institute of Health (NIH) developed a scoring system for HIES in which a score greater than 14 indicates a probable diagnosis. Our patient presented with recurrent multiple abscesses on her scalp, recalcitrant eczema, candida onychomycosis, alopecia universalis, and highly elevated levels of serum IgE. Using the NIH scoring system, a 30 total-point score in this patient indicated the likelihood of carrying the HIES genotype. To our knowledge, there are no specific treatments of HIES. The humanized recombinant monoclonal antibody against IgE, subcutaneous omalizumab, was successfully used in this patient.
Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand. sileena@staff1.mahidol.ac.th
Leena
L
Chularojanamontri
Saranjit
S
Wimoolchart
Papapit
P
Tuchinda
Kanokvalai
K
Kulthanan
Nisa
N
Kiewjoy
D000328:Adult; D000888:Antibodies, Anti-Idiotypic; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized; D002175:Candida / Q000276:immunology* / Q000472:pathogenicity; D003937:Diagnosis, Differential; D004802:Eosinophilia; D004890:Erythema; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007279:Injections, Subcutaneous; D007589:Job Syndrome / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D008218:Lymphocytosis; D000069444:Omalizumab; D014009:Onychomycosis / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D011537:Pruritus; D012867:Skin / Q000187:drug effects* / Q000276:immunology / Q000473:pathology
D000888:Antibodies, Anti-Idiotypic; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000069444:Omalizumab; D007073:Immunoglobulin E