The hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder
characterized by high serum levels of immunoglobulin E (IgE), recurrent cutaneous
and pulmonary infections, chronic dermatitis and a variety of connective tissue and
skeletal abnormalities. These patients share characteristic facial appearance and
many oral manifestations. We report a case of hyper IgE syndrome (HIES) also known
as “Job syndrome“.
Keywords
Hyper-immunoglobulin E syndrome - Job syndrome - Primary immunodeficiency - Eosinophilia
- Recurrent infections