A patient with a primary immunodeficiency may develop infections with Pseudomonas aeruginosa.
Primary immunodeficiencies associated with infections with Pseudomonas aeruginosa:
(1) CD40 ligand (CD154) deficiency (X-chromosomal recessive hyper IgM syndrome)
(2) CD40 deficiency (autosomal recessive hyper IgM syndrome)
Clinical manifestations may include:
(1) oral ulcer
(2) severe cutaneous infection, including ecthyma gangrenosum
(3) sepsis
