eMedicine Specialties > Dermatology > Pediatric Diseases

Chronic Granulomatous Disease: Follow-up

Author: Roman Janusz Nowicki, MD, PhD, Associate Professor, Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland
Contributor Information and Disclosures

Updated: May 29, 2009

Follow-up

Further Outpatient Care

  • Skin hygiene is an important element of further outpatient care for chronic granulomatous disease (CGD). The skin should be washed twice daily with a disinfectant soap.
  • The fingernails should be cut short.
  • The patient should be monitored for the results of antibacterial and antifungal prophylaxis.

Deterrence/Prevention

  • Chronic granulomatous disease in adults may be more common than previously assumed.
  • Because timely treatment, infection prophylaxis, and genetic counseling for affected families are possible, chronic granulomatous disease should be excluded in any patient with unexplained infections or granulomas.

Complications

  • Invasive aspergillosis and candidiasis may occur.
    • Patients with chronic granulomatous disease can be sensitized to Aspergillus species.
    • Allergic bronchopulmonary aspergillosis can develop.
  • Gastrointestinal complications include the following:
    • Enteritis and/or colitis
    • Crohn disease
    • Gastric outlet obstruction
    • Chronic gastrointestinal inflammation
  • Rheumatologic disorders include the following:
    • Discoid lupus erythematosus
    • Systemic lupus erythematosus
    • Raynaud syndrome
    • Nodular vasculitis
    • Juvenile rheumatoid arthritis
    • Immune-mediated thrombocytopenia
  • Other complications include the following:
    • Chorioretinitis
    • Obstruction of the urinary tract
    • Severe aphthous stomatitis
    • Granulomatous cheilitis

Prognosis

  • The long-term survival of patients in whom symptoms appear after they are aged 1 year is significantly better than that of patients whose illness starts in infancy.
  • Survival rates are variable but improving; approximately 50% of patients survive to age 30-40 years.
  • Infections are less common in adults than in children, but the propensity for severe life-threatening bacterial infections persists throughout life.

Patient Education

  • Good hygiene of the skin is an important element of treatment because the skin is a common portal of entry in serious infections.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make the diagnosis is a pitfall because the granulomas can occlude vital structures, especially those in the gastrointestinal and genitourinary systems.
  • Recurrent infections in the lungs, visceral lymph nodes, liver, and bones may lead to morbidity and mortality.

Special Concerns

  • The NBT test has been used for the prenatal diagnosis of chronic granulomatous disease (see Lab Studies).
 


More on Chronic Granulomatous Disease

Overview: Chronic Granulomatous Disease
Differential Diagnoses & Workup: Chronic Granulomatous Disease
Treatment & Medication: Chronic Granulomatous Disease
Follow-up: Chronic Granulomatous Disease
References
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Further Reading

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Keywords

chronic granulomatous disease, CGD, fatal granulomatosis of childhood, immunodeficiency disorders, X-linked recessive disorder, gp91phox, p22phox, p67phox, Xp21.1, Rac2, impaired phagocytic function, defective cytochrome b function, oxidase absence, oxidase malfunction

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Contributor Information and Disclosures

Author

Roman Janusz Nowicki, MD, PhD, Associate Professor, Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland
Roman Janusz Nowicki, MD, PhD is a member of the following medical societies: American Academy of Dermatology, European Academy of Dermatology and Venereology, and International Society for Human and Animal Mycology
Disclosure: Nothing to disclose.

Medical Editor

Jacek C Szepietowski, MD, PhD, Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland
Disclosure: Stiefel Salary Employment; Orfagen Consulting fee Consulting; Maruho Consulting fee Consulting; Astellas Consulting fee Consulting

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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