Progressive Fatal Cutaneous Gangrene Associated with Hypogammaglobulinemia

In 1952 Bruton¹reported the first known case of agammaglobulinemia. With newer refinements in methods of analysis of serum proteins, a clearer concept of this syndrome has developed. In brief, the clinical features of agammaglobulinemia or hypogammaglobulinemia are: Extreme susceptibility to infection Low to absent γ-globulin in the serum Immunologic unresponsiveness to antigenic challenge Frequent clinical improvement of symptoms with γ-globulin therapy. Physical examination usually reveals no abnormalities, although there may be a generalized decrease in lymphoid tissue and lack of regional lymphadenopathy in response to local infection.² Because of the lack of specificity of signs and symptoms, only a high index of suspicion and the use of appropriate laboratory studies can lead to the correct diagnosis. On routine determination of total serum proteins a globulin of 1 gm. % or less is suggestive of hypogammaglobulinemia. The diagnosis can be confirmed by serum electrophoresis or immunochemical analysis. Other laboratory findings