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,...

Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and...

common variable immunodeficiency (cvid) is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. here a 16-year-old boy is presented who was referred to our center with cough, dyspnea,cyanosis, and history of recurrent pneumonia. the diagnosis of cvid was made according to reduction all serum immunoglobulin levels...

Major histocompatibility complex (MHC) class II deficiency is a primary immunodeficiency disease characterized by abnormality of MHC class II molecules surface expression on peripheral blood lymphocytes and monocytes. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections but the immunodeficiency is not as severe as SCID (severe combined immunodeficien...

immunodeficiency and autoimmune disease may occur concomitantly in the same individual. some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. persistent antigen stimulation due to an inherent defect in the ability of the immune syst...