Autoinflammatory diseases are characterized by more or less spontaneous inflammation without inciting infection or autoimmunity. These can be either acquired or genetically determined. The latter –hereditary– autoinflammatory syndromes have been classified by some as primary immunodeficiencies: defects affecting the control of the innate arm of the immune system. Immunodeficiency syndromes, how...

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...

The following conditions and diseases that are associated with primary immunodeficiency disorder include, Combined variable immunodeficiency disease, Ataxia-telangiectasia, Chediak-Higashi syndrome, Complement deficiencies, DiGeorge syndrome, Hypogammaglobulinemia, Job syndrome, Leukocyte adhesion defects, Bruton disease, Congenital agammaglobulinemia, Selective deficiency of IgA, Wiscott-Aldri...

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...

E osinophilia, defined as greater than 450 eosinophils/ L, can occur in various disease processes, including infectious, allergic, neoplastic, and primary hematologic disorders. Marked eosinophilia ([1500 eosinophils/ L) can be a diagnostic dilemma because blood eosinophilia rarely exceeds 1500/ L in atopic/allergic diseases. Hyperimmunoglobulinemia E is seen in allergic, parasitic, and primary...

There are many challenges to those who specialize in the research, education, and clinical care of patients with primary immunodeficiency. The first of these difficult challenges is just keeping up with this rapidly moving area of medicine, a task that is obvious to those who are engaged in the ongoing discoveries of genetic mutations causing previously unrecognized primary immunodeficiencies. ...

Introduction With increased diagnostic capabilities and treatment modalities in the field of primary immunodeficiencies (PID), many pediatric patients survive beyond childhood experience a change care to adult-oriented healthcare system. Unfortunately, transition pathways for PID are less clearly defined, resulting deterioration quality adulthood. Hence, this is first regional study address cli...