JOB'S SYNDROME: A RARE CASE REPORT-Reference-Cited by-同舟云学术

JOB'S SYNDROME: A RARE CASE REPORT

Published:2021-08-01 Issue: Volume: Page:34-35
ISSN:
Container-title:INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH
language:en
Short-container-title:ijsr

Author:

Rajesh Eshwar¹,T Shakuntala²

Affiliation:

1. Madras Medical College, Chennai, India.

2. Professor, Department of Paediatrics, Government Mohan Kumaramangalam Medical College & Hospital, Salem, India.

Abstract

Hyper IgE Syndrome (HIES) or Job's Syndrome is a complex primary immunodeciency disorder characterized by elevated serum IgE levels, recurrent skin rashes, eosinophilia, skeletal and connective tissue abnormalities. The patients also exhibit features of specic facies, retention of deciduous teeth and susceptibility to infections. HIES is a rare genetic disorder where some cases are inherited as autosomal dominant or autosomal recessive but most cases are supposedly sporadic. Hereby we present a case of 12-year-old male child admitted in the hospital with complaints of fever, skin rashes, intermittent abdominal pain and generalised lymphadenopathy. As there is no denite cure for HIES, the approach was directed towards conservative management. Due to the extreme paucity of reported cases in literature, this case report can help shed light on a syndrome of such rarity.

Publisher

World Wide Journals

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