Indian Journal of Dermatology
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Year : 2009  |  Volume : 54  |  Issue : 4  |  Page : 378-379

Hyper IgE syndrome with umbilical hernia

Department of Dermatology, Dr. B.R. Ambedkar Medical College and Hospital, K.G. Halli, Bangalore - 560 045, Karnataka, India

Correspondence Address:
Prabhakar M Sangolli
# 8, 11th Cross, Gayathrinagar, HBCS Layout, Basaveshwaranagar, Bangalore - 560 079
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.57619

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Hyper IgE Syndrome (HIES) is a rare multi system genetic immunodeficiency disorder, with immunological and non-immunological features. Immunolgical features are 1) Recurrent cutaneous abscesses, 2) Atopic dermatitis like lesions, 3) Sino pulmonary infections, 4) Elevated serum IgE levels and 5) Abnormal neutrophil chemotaxis. Non immunological features include cranio facial and skeletal abnormalities. We are reporting a girl with classical features of HIES with umbilical hernia with her younger brother suffering from right sided inguinal hernia, as both herniae are hitherto unreported in patients with HIES.

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