Hyper IgE syndrome with umbilical hernia.

Indian Journal of Dermatology
N C HiremathN Soumya

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.

References

Mar 4, 1999·The New England Journal of Medicine·B GrimbacherJ M Puck
Jan 15, 2004·The Journal of Pediatrics·Eleonore D RennerBodo Grimbacher

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Methods Mentioned

BETA
ESR
biopsy

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