Dermatologic Manifestations of Juvenile Xanthogranuloma 

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

Background

Juvenile xanthogranulomas (JXGs) are benign; usually asymptomatic; self-healing; red, yellow, or brown papules and nodules composed of histiocytic cells that predominantly occur in infancy and childhood. Papules or nodules occur in the skin, eyes, and viscera. JXG is the most common form of non–Langerhans cell histiocytosis.

See the image below.

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

Adamson first reported JXG in the English literature in 1905. He presented a child who developed numerous yellow-white papules on the body in the first 2 weeks of life. He named the entity congenital xanthoma multiplex.

In 1912, McDonagh presented the first case review and renamed the condition nevoxanthoendothelioma (although the condition is not associated with nevi or endothelial cells). In 1954, Helwig and Hackney again retermed it juvenile xanthogranuloma, reflecting its histopathologic appearance. Laurb and Lain first reported JXG with visceral involvement in 1937. Blank et al first described ocular involvement in 1949.

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Pathophysiology

The etiology of juvenile xanthogranuloma (JXG) is not fully known. The papules and nodules of JXG represent collections of differentiated non–Langerhans cell histiocytes. The consensus is that the cells of origin are dermal dendrocytes. As postulated, JXG may be a granulomatous reaction of histiocytes to an unidentified stimulus, possibly of either physical or infectious etiology. Evidence from Kraus et al,[1] however, suggests a possible CD4+ plasmacytoid monocyte origin. Inhibition of cellular apoptosis appears to play a minor role in the growth of xanthogranulomas.[2]

The appearance of giant cells and foamy lipid-laden histiocytes generally occurs late and apparently is a secondary event, possibly in response to cytokine production by histiocytes. Serum lipid levels are normal and remain normal.

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Epidemiology

Race

Juvenile xanthogranuloma (JXG) occurs in whites approximately 10 times more frequently than in African Americans.

Sex

In childhood, juvenile xanthogranuloma (JXG) occurs predominately in males (1.4:1). Equal incidence occurs in adult males and females. Multiple cutaneous lesions occur predominantly in males (12:1).

Age

Approximately 35% of cases of juvenile xanthogranuloma (JXG) occur at birth, with as many as 71% of cases occurring in the first year. The mean age at presentation is 22 months. Most JXGs resolve by age 5 years. Despite the term juvenile in the disease name, 10% of cases manifest in adulthood.

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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.

References

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