Cyclosporin for treatment of Hyperimmunoglobulin E Syndrome: A case report

Zakiudin Munasir; Teti S Gunarti; Nia Kurniati; Arwin AP Akib; Dina Muktiarti

doi:10.35582/ijcpch.v1i1.6

Zakiudin Munasir Departement of Pediatric, Faculty of Medicine, Universitas Indonesia
Teti S Gunarti Department of Child Health, Faculty of Medicine University of Indonesia, Indonesia
Nia Kurniati Department of Child Health, Faculty of Medicine University of Indonesia, Indonesia
Arwin AP Akib Department of Child Health, Faculty of Medicine University of Indonesia, Indonesia
Dina Muktiarti Department of Child Health, Faculty of Medicine University of Indonesia, Indonesia

DOI: https://doi.org/10.35582/ijcpch.v1i1.6

Keywords: Job’s syndrome, cyclosporine therapy, Hyperimmunoglobulin E Syndrome (HIES)

Abstract

Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by elevated serum IGE, dermatitis, and recurrent skin and lung infections. The pathogenesis of this disease is still unknown. There is no definitive therapy for HIES, the treatment is mainly for prevention and management of infections. Immunoglobulin supplementation or low dose cyclosporine has been reported helpful. We present a case of 16-month-old boy with hyperimmunoglobulin E syndrome. He was administrated with 3 grams (300 mg/kgBW) of intravenous immunoglobulin. After IVIG administration the papules decreased in number or partially resolve but fever and itchy rash still persisted. The patient then received cyclosporine 2x25mg/day (5mg/kgBW/day) for 6 months. There was improvement in clinical manifestations after 1 month therapy with cyclosporine, although IgE level did not decreased. Two years after treatment, the patient is still in good condition