Volume : VII, Issue : IV, April - 2017

JOBS SYNDROME A RARE CASE REPORT

Dr Nita R Sutay, Dr Devanand Gulab Chaudhari, Dr. Yugesh Kumar Agrawal

Abstract :

 Background

 

Job’s syndrome (Hyper IgE Syndrome (HIES) or Buckley syndrome) is a rare primary immunodeficiency syndrome characterized by recurrent severe staphylococcal infections like skin abscesses, severe lung infections that result in pneumatoceles and multi organ dysfunction. The hallmark finding in Job’s syndrome is very high concentrations of serum IgE. Incidence is less than 1 per million people. We report here a case of 1 1/2 year old female child who had history of swelling of left thigh following a thorn prick. She was diagnosed to be having Job’s syndrome on the basis of classical triad ie Raised serum IgE, recurrent staphylococcal abscesses and recurrent respiratory infections.

 

Case Report:

 

We present here a case of 1 1/2 year old female child who had history of swelling over left thigh following a thorn prick. After this the child gradually developed multiple swellings all over the body. There was also history of fever and peeling of skin involving multiple sites. On examination the child was malnourished and stunted. There were multiple fluctuant swellings over chest and abdomen with ulceration over left thigh. Multiple abscesses over knee, left wrist and elbow joints with restricted, painful movements of involved joints were also seen. Skin biopsy was suggestive of features of atopic dermatitis. The diagnosis of Jobs syndrome was made on the basis of presence of staphylococcal abscesses, history of recurrent respiratory tract infections and finally demonstration of abnormally high serum IgE levels.

 

 

 

 

 

Conclusion:–

 

The classical triad of recurrent respiratory tract infections, multiple abscesses and raised IgE levels is diagnostic of Jobs syndrome. Any patient having these signs and symptoms should be investigated by estimation of serum IgE levels for confirmation or ruling out Job’s syndrome. Recurrent respiratory tract infections and pulmonary complications are the common causes of mortality in these children. Late diagnosis is associated with growth abnormalities. Early diagnosis and institution of prophylactic treatment reduces complications and ensures proper growth.

Keywords :

Article: Download PDF   DOI : 10.36106/ijar  

Cite This Article:

Dr Nita R Sutay, Dr Devanand gulab Chaudhari, Dr. Yugesh Kumar Agrawal, JOBS SYNDROME A RARE CASE REPORT, INDIAN JOURNAL OF APPLIED RESEARCH : Volume‾7 | Issue‾4 | April-2017

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