hyper - immunoglobulin e syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum ige associated with peculiar face and skeletal features. we report a seven-year old  girl  presenting with persistent productive cough and  history of chronic eczematoid facial lesions since infancy  and  two episod...

Background: The etiology of urticaria is recognized only in a minority of cases. Allergies to a wide variety of agents have been suspected, as well as hyper reactivity to food additives or drugs. Recently, autoimmune reaction to immunoglobulin E (IgE) and later to high-affinity IgE receptors on mast cells has been reported. The aim of this study was to show elevated serum IgE levels in the pati...

Asthma and other respiratory diseases have increased in the last years among Venezuelan children from helminthic endemic areas where the infection by Ascaris lumbricoides has been associated to bronchial airway inflammation in parasitized individuals. The aim of this work was to investigate the possible associations between the development of bronchial hyper reactivity and the immune response a...

Kidney disease due to Aspergillus fumigatus is a rare finding in hyper-IgE syndrome. We report a patient with autosomal dominant hyper-IgE syndrome, recurrent pneumonia, and acute necrosuppurative pyelonephritis caused by Aspergillus fumigatus with a fatal outcome. We emphasize the severity and the difficulties in the management of renal complications that could be limiting the survival of thes...

Immigrants from undeveloped countries are a growing problem in Europe. Spain has become a frequent destination for immigrants (20% of whom are children) because of its geographic location and its historic and cultural links with Africa and Latin America. Eosinophilia is frequent in adult immigrants, travelers and expatriates coming from tropical areas. However, there are few studies that focus ...

Over the last 4 years, three genetic etiologies of hyper IgE syndromes have been identified: STAT3, DOCK8, and Tyk2. All of these hyper IgE syndromes are characterized by eczema, sinopulmonary infections, and greatly elevated serum IgE. However, each has distinct clinical manifestations. Mutations in STAT3 cause autosomal dominant HIES (Job's syndrome), which is unique in its diversity of conne...

kimura’s disease (kd) is a chronic inflammatory disorder primarily seen in male asians during the second and third decades of life. clinically, it presents as solitary or multiple subcutaneous nodules, predominantly in the head and neck region, typically in the pre auricular region, forehead, and scalp. the etiology of kimura disease is still unknown. this disorder should be suspected when the ...