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October 1960

Progressive Fatal Cutaneous Gangrene Associated with Hypogammaglobulinemia

Author Affiliations

Chapel Hill, N.C.

From the Division of Dermatology, Department of Medicine (Donald C. Abele and Richard L. Dobson), Department of Surgery (James F. Newsome), and Department of Pathology (A. S. Lineberger), University of North Carolina School of Medicine.

Arch Dermatol. 1960;82(4):565-569. doi:10.1001/archderm.1960.01580040083014

In 1952 Bruton1 reported 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:

  1. Extreme susceptibility to infection

  2. Low to absent γ-globulin in the serum

  3. Immunologic unresponsiveness to antigenic challenge

  4. 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

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