Adaptive Immune System

The main immunologic defect is a failure to produce gamma interferon by helper T cells (Th1 pathway), which reduces the ability of macrophages to kill bacteria. This leads to an increase in Th-2 cells and, as a consequence, a high IgE level
Cold abscess (staph common)
The increased IgE leads to increased histamine release. Histamine blocks certain aspects of the inflammatory response, hence the cold abscesses.
Histamine also inhibits neutrophil chemotaxis, another feature of this syndrome.
Review of Medical Microbiology and Immunology, 13e
http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/hyper-ige-syndrome/

In response of infection (like yeastB-glucan binding to dectin-1) transforms CD4+ T cell to Th17 by a STAT 3 dependent process
Th17 cell procudes IL -22 and IL-17. Together, IL-22 and IL-17 work to generate DEFENSINS in the epithelial tissue
STAT 3 mutation can lead to the defective interaction causing decreased production of DEFENSINS
Defect in Leucocyte activation in the epithelium mediated through IL-17 leads to mucocutaneous infection

J Allergy Clin Immunol 2010;125:S33-40.

How I treat severe combined immunodeficiency Blood 2013
How I treat ADA-SCID deficiency Blood 2009
B-Cell

Impaired Development (Agammaglobunemia)
Impaired Functioning (Hyper IgM, CVID, IgA deficiency)
How I treat common variable immune deficiency Blood 2010
Recognition, clinical dx. and mnt of pts. with primary antibody deficiencies: a systematic review Cli Exp Imm 2007
Laboratory evaluation of B Cell Function

Selective Antibody Deficiency

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