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Chronic granulomatous disease in Infant/Neonate
Other Resources UpToDate PubMed

Chronic granulomatous disease in Infant/Neonate

Contributors: Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Cynthia Christy MD
Other Resources UpToDate PubMed

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.

Codes

ICD10CM:
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
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Chronic granulomatous disease in Infant/Neonate
A medical illustration showing key findings of Chronic granulomatous disease : Diarrhea, Abscess, Short stature
Clinical image of Chronic granulomatous disease - imageId=459553. Click to open in gallery.  caption: 'A deep pink nodule with surrounding erythema on the leg.'
A deep pink nodule with surrounding erythema on the leg.
Copyright © 2024 VisualDx®. All rights reserved.