Correct!
1. Hyperimmunoglobulin E Syndrome

There is no evidence of myeloma in this patient. Kimura disease is a disorder of unknown etiology associated with lymphadenopathy. Strongyloidiasis is a chronic infection with the roundworm often seen in the Southeast United States. Wiskott-Aldrich syndrome is a rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhea (secondary to the thrombocytopenia).

Hyperimmunoglobulin E syndrome (HIES) is also know as Job's syndrome. The name Job's syndrome is taken from the following bible passage:

"So went Satan forth from the presence of the LORD and smote Job with sore boils from the sole of his foot unto his crown.” - King James Bible

In 1966 HIES was first described and characterized by eczematoid dermatitis and recurrent skin and pulmonary infections (1). In 1972 the association with elevated IgE was first appreciated. Clinical features of HIES include the following:

• Eczema – 100%
• Peak serum IgE > 2,000 kIU/mL - 97%
  
• Eosinophilia - 93%
  
• Boils – 87%
  
• Recurrent pneumonias – 87%
  
• Characteristic facies – (prominent forehead, facial asymmetry) - 83%
  
• Scoliosis – 63%
  
• Coronary aneurysms – 37%

Which of the following signal transduction pathways has been identified as the cause of HIES? (Click on the correct answer to proceed to the next panel)

1. G Protein-coupled signal transduction
   
2. RAS-mitogen activated protein kinase signal transduction
   
3. STAT3 signal transduction
   
4. Transforming growth factor-β (TGF-β) signal transduction
   
5. Toll-like receptor TLR3 signal transduction

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