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...

patients with organic acidemia are prone to different infections, which lead to acidosis episodes. some studies have evaluated the status of immune system in acidotic phase in these patients, but to the best of our knowledge no study has evaluated the immune system in non-acidotic phase of the disease. in this study, thirty-one patients with organic acidemia were enrolled. for evaluation of hum...

Primary immunodeficiency diseases (PIDs) is a diverse group of diseases, characterized by a defect in the immune system. These patients are susceptible to recurrent respiratory infections, gastrointestinal problems, autoimmune diseases, and malignancies. In most cases, patients with primary immunodeficiency disorders have genetic defects and are monogenic disorders that follow a simple Mendelia...

recurrent and chronic infections in children are one of the most common reasons for physicians' visits that make a diagnostic challenge to pediatricians. although the majority of referred  children  with  recurrent  infections  are  normal,  underlying causes  of  recurrent infection  such  as  atopy,  anatomical and  functional  defects,  and  primary or  secondary immunodeficiency must be con...

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...

the primary immunodeficiency (pi) disorders are abnormalities in development and maturation of the immune system. individuals with pi disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. in this survey we compared health-related quality of life of primary immune deficient patients with healthy childr...

patients  with  primary  antibody  deficiencies  (pad)  are  susceptible  to  recurrent  and chronic infections and a variety of complications. this study was performed to assess quality of life (qol) of pad patients who were under long term treatment and regular follow-up.thirty six adults with proved diagnosis of pad, who had received regular intravenous immunoglobulin replacement therapy, we...