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...

primary antibody deficiencies are the most frequent primary immunodeficiency disorders. bronchiectasis as a feature of these disorders may be developed due to some factors such α-1-antitrypsin deficiency. in order to determine the prevalence of two common α-1-antitrypsin deficiency alleles (pi*z and pi*s) in iranian patients with antibody deficiency, this study was performed. the prevalence of ...

Primary immunodeficiencies (PIDs) are genetic disorders that predispose to frequent and severe infections, autoimmunity and cancer. The expanded life span of such patients increases the overall risk for developing cancer, which is now estimated at 4-25% . The type of malignancy depends on the primary immunodeficiency, the age of the patient and possible viral infection, suggesting that differen...

Nine patients with immunoglobulin deficiency and various haematological disorders are presented. In all patients, recurrent infections had antedated the onset of the haematological disorder but, in most, the possibility of primary immunodeficiency had not been considered until after the haematological diagnosis had been established. The recognition of immunodeficiency is important since such pa...

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...