Anti-cytokines autoantibodies (ACAA) are types of against soluble immune factors and may be mirrored by autoimmune disease biomarkers. The manifestation secretion these antibodies is associated to primary immunodeficiency (PID), but also directly or indirectly related infectious occurrence, which could assumed as a consequence cytokine deficiency

Kaposi's sarcoma (KS) is a malignant vascular neoplasm that typically appears opportunistically in patients with acquired immunodeficiency syndrome (AIDS); it can also arise organ transplanted subjects and exceptionally carriers of autoimmune diseases, such as Behçet's disease. A 23-year-old man disease who debuts clinically histologically compatible dermatosis KS. Conclusions. KS not exclusive...

One in 2,000 children younger than 18 years is thought to have a primary immunodeficiency disease. Antibody, combined B-cell and T-cell, phagocytic, and complement disorders are the most common types. Children with these diseases tend to have bacterial or fungal infections with unusual organisms, or unusually severe and recurrent infections with common organisms. A family history of primary imm...

Evans syndrome, a combination of autoimmune hemolytic anemia and immune thrombocytopenia, is rare disease in children. In childhood, it may turn out to be one the first manifestations primary immunodeficiency or an dysregulation syndrome. Here we present clinical case patient who was initially diagnosed with syndrome did not respond well therapy. Based on results genetic testing, child then imm...

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a parti...

Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital. Method: All PID cases with a confirmed diagnosis,...

Arthritis may be the first clinical manifestation of primary hypogammaglobulinaemia. In 16 years of 281 patients who had immunodeficiency, 30 had arthritis at presentation. It was more common in Bruton's disease (15 (22%) of 69 patients) than in other forms of immunodeficiency (15 (7%) of 212 patients). Non-septic arthritis was more prevalent than septic arthritis, particularly monoarticular ar...