immunodeficiency 131

A primary immunodeficiency disease that is characterized by low IgM, IgG, and IgA serum levels, low plasma cell counts, abnormal T-cell subsets, and early graying of hair that has_material_basis_in heterozygous or homozygous mutation in the IRF4 gene on chromo

A primary immunodeficiency disease that is characterized by low IgM, IgG, and IgA serum levels, low plasma cell counts, abnormal T-cell subsets, and early graying of hair that has_material_basis_in heterozygous or homozygous mutation in the IRF4 gene on chromosome 6p25.

Signs and symptoms

• Spongiosis
• Decreased memory B cell proportion
• Increased memory CD8+ T cell proportion
• Increased memory CD4+ T cell proportion
• Increased CD21low B cell proportion
• Unusual molluscum contagiosum
• Increased transitional B cell proportion
• Premature graying of hair
• Inflammation of the large intestine
• BCGitis