Allergy and Airway: Student/Resident Case Report Poster - Allergy and Airway |

Three Unusual Presentations of Job's Syndrome (Hyper Immunoglobulin E Syndrome) FREE TO VIEW

Tanushree Gahlot, MD; Arup Basu, MD; Amit Dhamija, MD; Mayank Saxena, MD
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Lady Harding Medical College, New Delhi, India

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):21A. doi:10.1016/j.chest.2016.08.024
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SESSION TITLE: Student/Resident Case Report Poster - Allergy and Airway

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: First described in 1966 by Davis et al, Jobs Syndrome is a primary immunodeficiency disease presents as multisystem disorder characterized by triad of abscesses, pneumonia, and elevated IgE level.

CASE PRESENTATION: Here we present case series of three unusual presentation of Hyper Immunoglobulin E syndrome or HIES (Jobs syndrome). First case is of a middle aged female with recurrent spontaneous bacterial peritonitis and an eosinophilic pleural effusion. She had history of recurrent skin rashes and pustules since birth. Laboratory investigations revealed mild peripheral eosinophilia with high total Immunoglobulin E (IgE) : 9835 IU/ml. Second case is of another young female with a background of allergic bronchitis, recurrent allergies and chronic ulcers that presented with unusual thigh abscess and subsequently diagnosed with invasive pulmonary cryptococcosis. Laboratory investigations done showed markedly elevated high levels of Ig E(19,303 IU/ml). Third and the last case is of middle aged man who presented with pyopneumothorax not responding to medical management and underwent thoracoscopic decortication. His investigations revealed elevated IgE(4,200 IU/ml) and peripheral eosinophilia. Treatment in all three patients was directed at management of infections by systemic antibiotics/antifungals, drainage of pus and topical therapy for skin conditions.

DISCUSSION: The hyper-IgE syndromes(HIES) or JOB’s syndrome are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity like dermatitis, boils, pneumonia, elevated serum IgE levels. Spectrum of HIES has 2 distinct entities, the classical hyper-IgE syndrome (autosomal dominant) which presents with cluster of facial, dental, skeletal, and connective tissue abnormalities and the autosomal recessive hyper-IgE syndrome without these features. STAT3 gene mutation can identify patients with classic HIES. In this series we add on to the clinical spectra of Jobs Syndrome by presenting three unusual presentation of Jobs syndrome.

CONCLUSIONS: To conclude diagnosis in all 3 patients lead to detection of underlying immunodeficient state and need of long term care in terms of prolonged antibiotics, skin care, chest physiotherapy in these patient that improved the prognosis and quality of life in the patients.

Reference #1: Grimbacher B. Holland SM Puck JM. Hyper-IgE syndromes. Immunological reviews. 2005;203:244-50.

DISCLOSURE: The following authors have nothing to disclose: Tanushree Gahlot, Arup Basu, Amit Dhamija, Mayank Saxena

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