Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 18 2 2019 04 01 225 229 EN Samin Sharafian Department of Allergy and Clinical Immunology, School of Medicine, Bushehr University of Medical Sciences, Bushehr, Iran Masoud Movahedi Department of Allergy and Clinical Immunology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Arash Kalantari Department of Allergy and Clinical Immunology, Vali-e-Asr Hospital, Imam Khomeini Hospital Complexes, Tehran University of Medical Sciences, Tehran, Iran Nima Parvaneh Department of Allergy and Clinical Immunology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mohammad Gharagozlou Department of Allergy and Clinical Immunology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran 2018 01 25 2018 07 04 Hyperimmunoglobulin E syndrome (HIGE) is considered as a phagocytic or a newly classified complex and heterogeneous primary immunodeficiency disease with symptoms such as increased levels of immunoglobulin E, eczema, and, recurrent lung and skin infections. In this paper, we have presented a rare case of this syndrome. A 9-year-old Iranian girl presented with a history of pruritic maculopapular rash who was eventually diagnosed as a case of HIGE. In her recent admission, she had dysphonia, stridor and huge cauliflower cutaneous lesions on her neck, finger and vocal cords, which did not respond to intravenous antibiotics, and ultimately required surgical removal. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/1836 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/1836/1403