X-linked agammaglobulinemia: a review of literature and a case report

X-linked agammaglobulinemia (XLA), or Bruton’s agammaglobulinemia, – is a primary immunodeficiency, caused by defects in the BTK gene encoding tyrosine kinase. The lead to arrest of B-lymphocyte development and, as result, agammaglobulinemia. disease manifests with recurrent infections starting infancy. gold standard XLA treatment intravenous subcutaneous immunoglobulin substitution proved effective various multicenter studies and increases quality life patients. However, there are cases delayed verification, untimely treatment, which leads severe, life-threatening conditions. We present review literature case report an patient ecthyma gangrenosum. patient's parents gave consent use their child's data, including photographs, for research purposes publications.