Hyper-IgE Syndrome: A Rare Entity
Received on: October 03, 2017, Accepted on: October 11, 2017, Published on: October 25, 2017
Citation:Fehmida Najmuddin (2017) Hyper-IgE Syndrome: A Rare Entity. SF AIDS HIV Res J 1:1.
Copyright: ©2017 Fehmida Najmuddin. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Serum IgE Levels
We started the child on broad spectrum antibiotics, anti-helminthic and supportive therapy, following which the child did not improve clinically and repeat CBC showed persistent eosinophilia. The recurrent skin lesions were diagnosed as atopy dermatitis and therapy for the same was initiated.
With the above clinical picture and investigations, patient was diagnosed as Hyper IgE syndrome, counseled for bone marrow transplantation and as in on regular follow up.
The autosomal dominant form of HIS is characterized by recurrent pneumonia, sinopulmonary and fungal infections as compared to the AR form which is more commonly associated with skin, food allergies and Asthma. AD forms are strongly associated with history of musculoskeletal involvement and coarse facies which is absent in the AR form [4, 5].
Since tuberculosis and Acquired Immunodeficiency syndrome (HIV) is endemic in our country, our initial line of investigation was to rule out the above causes. The child also had recurrent atopic dermatitis and pneumonia, which further compelled us to investigate for common allergic cascade of diseases and treatment was initiated for the same. Since the patient did not have any relief in symptomatology and had repeated episodes of pneumonia and skin infections in the form of areolar abscess, we suspected immunodeficiency disorder, and on investigations, diagnosed the child with Hyper IgE syndrome in view of very high levels of Serum IgE (˃ 2000 IU/ml).
Our patient was having clinical manifestations which were likely of an AR form of HIS. Genetic mutational analysis could not be done due to financial constraints.
The treatment of AD HIS is a long term administration of anti-staphylococcal antibiotics and Intravenous Immunoglobulin [6, 7]. The treatment of choice in AS HIS is allogenic bone marrow transplantation  and has a poorer prognosis as compared to the AD HIS.
With the above report we want to highlight the rarity of this syndrome, the need for a high index of suspicion particularly in those children who have allergic symptoms and don?t respond to the therapy with recurrent pneumonia. Lastly though high serum IgE levels are associated with allergic cascade, one should always rule out Hyper IgE syndromes if the levels are above 2000 IU/ ml with characteristic clinical features.