Chronic granulomatous disease

Alternative names:

CGD

Definition:

An inherited abnormality of certain cells of the immune system that "ingest" bacteria and kill them (phagocytic cells); the abnormality results in chronic infection by certain types of bacteria.

Causes, incidence, and risk factors:

Chronic granulomatous disease (CGD) is transmitted as a sex-linked recessive trait. CGD is almost universally a disease affecting males, although it is possible for a woman to have two affected X chromosomes and subsequently develop CGD.

In this disease, the inability of phagocytic cells to kill certain bacteria and fungi leads to long term (chronic) and repeated (recurrent) infections appearing in the first years of life. Milder forms may appear in adolescence.

Impetigo, skin abscesses and furuncles, and perianal and rectal abscesses are common. Recurrent pneumonia is a significant problem and may be caused by bacteria not typically found in most pneumonias. Chronic swelling of the lymph nodes in the neck with abscess formation is common.