Chronic granulomatous disease in Infant/Neonate
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Synopsis
Chronic granulomatous disease (CGD) is the result of a rare inherited selective immunodeficiency (impaired intracellular microbial killing) that prevents the clearing of certain bacteria and fungi. (Aspergillus has been reported.) It has two forms: a more common and more severe X-linked type seen only in boys, and an autosomal recessive type.
Recurring granulomatous and purulent infections begin early in life, persist, and lead to early death if the disease is not treated aggressively.
The disease can begin with findings of dermatitis and lymphadenitis in the infant. Subcutaneous abscesses, furunculosis, and suppurative panniculitis are all findings in this disease.
Systemic symptoms include persistent diarrhea, osteomyelitis, chronic urinary tract infections, pericarditis, and liver and lung granulomatous involvement. In older children, acne vulgaris can be severe.
Recurring granulomatous and purulent infections begin early in life, persist, and lead to early death if the disease is not treated aggressively.
The disease can begin with findings of dermatitis and lymphadenitis in the infant. Subcutaneous abscesses, furunculosis, and suppurative panniculitis are all findings in this disease.
Systemic symptoms include persistent diarrhea, osteomyelitis, chronic urinary tract infections, pericarditis, and liver and lung granulomatous involvement. In older children, acne vulgaris can be severe.
Codes
ICD10CM:
L92.8 – Other granulomatous disorders of the skin and subcutaneous tissue
SNOMEDCT:
387759001 – Chronic granulomatous disease
L92.8 – Other granulomatous disorders of the skin and subcutaneous tissue
SNOMEDCT:
387759001 – Chronic granulomatous disease
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Last Updated:03/05/2024
Chronic granulomatous disease in Infant/Neonate