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Chronic granulomatous disease - Skin in Child
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Chronic granulomatous disease - Skin in Child

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Contributors: Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
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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.

For more information on the X-linked type, see OMIM.

Codes

ICD10CM:
L92.8 – Other granulomatous disorders of the skin and subcutaneous tissue

SNOMEDCT:
387759001 – Chronic granulomatous disease

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Last Updated: 03/29/2017
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Chronic granulomatous disease - Skin in Child
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Chronic granulomatous disease : Diarrhea, Short stature
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