Dermatologic Manifestations of Juvenile Xanthogranuloma Follow-up

  • Author: Caroline DS Piggott, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 24, 2012
 

Further Outpatient Care

  • Follow-up visits may be scheduled at regular intervals for reassurance and to monitor associated complications. For patients with juvenile xanthogranulomas (JXGs) and type 1 neurofibromatosis (NF1), physicians should watch for signs and symptoms of juvenile myelomonocytic leukemia (JMML).[22]
  • Patients with ocular involvement should be checked regularly by an ophthalmologist to prevent rare complications, such as glaucoma.[23]
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Complications

  • Complications associated with juvenile xanthogranulomas (JXGs) are rare and are dependent on the site of involvement and associated conditions.
  • Ocular involvement may progress to ocular hemorrhage, glaucoma, or retinal detachment. These complications are best prevented through early detection.
  • CNS involvement is a very rare complication.
  • Hepatic failure is a rare, but potentially fatal, complication of systemic JXG.[24]
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Prognosis

  • In the absence of therapeutic intervention, juvenile xanthogranulomas (JXGs) flatten with time. Both cutaneous and extracutaneous lesions involute spontaneously within 3-6 years.
  • Hyperpigmentation, mild atrophy, or anetoderma may persist.
  • Lesions can recur after resection. The relapse rate is approximately 7%.
  • In the absence of neurofibromatosis, no systemic health implications are involved, with a few rare exceptions.
  • Vigilantly screen patients with neurofibromatosis and JXG for leukemia.
  • Ocular, neurologic, and hepatic disease are rare but may have serious long-term consequences.
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Patient Education

  • Reassure patients and their families.
  • Instruct patients concerning associations related to clinical situations (neurofibromatosis, ocular findings in diffuse JXG, JMML), and direct patient education toward these conditions.
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Contributor Information and Disclosures
Author

Caroline DS Piggott, MD  Resident, Department of Medicine, Division of Dermatology, University of California, San Diego

Disclosure: Nothing to disclose.

Coauthor(s)

Michael L Smith, MD  Associate Professor of Medicine (Dermatology) and Pediatrics, Vanderbilt University School of Medicine; Consulting Staff, Vanderbilt University Hospital

Michael L Smith, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Hunter H Sams, MD  Consulting Staff, Denver Dermatology Consultants, PC

Hunter H Sams, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Jean Paul Ortonne, MD  Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France

Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

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Smooth, domed, yellow-brown, 5-mm papule on right arm of a 6-month-old boy.
 
 
 
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