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Indian J Dermatol. 2009;54(4):378-9. doi: 10.4103/0019-5154.57619.

Hyper IgE syndrome with umbilical hernia.

Indian journal of dermatology

N C Hiremath, N T Madan Mohan, C Srinivas, Prabhakar M Sangolli, K Srinivas, N Soumya

Affiliations

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

PMID: 20101344 PMCID: PMC2807719 DOI: 10.4103/0019-5154.57619

Abstract

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.

Keywords: Hyper IgE syndrome; inguinal hernia; umbilical hernia

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