Juvenile Xanthogranuloma 

  • Author: Theodore Curtis, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 26, 2010
 

Background

Juvenile xanthogranuloma (JXG) primarily is a self-limited dermatologic disorder that is associated rarely with systemic manifestations. Infants and small children are mainly affected.

JXG consists of lesions that may be single or multiple and appear as firm, slightly raised papulonodules several millimeters in diameter. They are tan-orange in color and occur frequently on the head and neck, but many extracutaneous sites have been reported.

The eye, particularly the uveal tract, is the most frequent site of extracutaneous involvement. Approximately one half of patients with ocular involvement have skin lesions. JXG is the most frequent cause of spontaneous hyphema in children and can result in secondary glaucoma and eventual blindness.

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Pathophysiology

The etiology of JXG is unknown. JXG is believed to result from a disordered macrophage response to a nonspecific tissue injury, resulting in a granulomatous reaction. JXG is on a spectrum of histiocytic disorders that includes benign cephalic histiocytosis, generalized eruptive histiocytosis, adult xanthogranuloma, and progressive nodular histiocytosis. These diseases are less common than the related Langerhans cell histiocytoses.

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Epidemiology

Frequency

United States

The frequency is unknown, but it may be higher than reported, since lesions occur early in life, may be misdiagnosed, and spontaneously regress. In those affected, 92% of ocular involvement occurs before age 2 years.[1]

Mortality/Morbidity

Cutaneous lesions generally are self-limited and rarely require treatment. The risk of morbidity is high with ocular involvement and can include hyphema, glaucoma, corneal blood staining, cataract, vascular occlusion, and retinal detachment, all of which can lead to amblyopia in childhood. Rarely, death has been reported among children with visceral JXG.

Race

No reported predilection of race exists in JXG, although few African American patients have been described.

Sex

Cutaneous JXG is reported 1.5 times more in male children, but no sex predilection exists in adults. Both sexes are equally at risk for ocular involvement.

Age

JXG may be present at birth (in about 10%) but most often arises in infancy. Children younger than 6 months are more likely to have multiple lesions.[1] Zimmerman reported 64% of cutaneous lesions to be present by age 7 months and 85% before 1 year. Adult onset is reported infrequently.

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Contributor Information and Disclosures
Author

Theodore Curtis, MD  Assistant Professor, Department of Ophthalmology, University of Colorado; Consulting Staff, Rocky Mountain Lions Eye Institute

Theodore Curtis, MD is a member of the following medical societies: American Academy of Ophthalmology and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Coauthor(s)

David T Wheeler, MD  Associate Professor, Departments of Ophthalmology and Pediatrics, Oregon Health & Science University

David T Wheeler, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Cypel TK, Zuker RM. Juvenile xanthogranuloma: Case report and review of the literature. Can J Plast Surg. Fall 2008;16(3):175-7. [Medline]. [Full Text].

  2. Liang S, Liu YH, Fang K. Juvenile xanthogranuloma with ocular involvement. Pediatr Dermatol. Mar-Apr 2009;26(2):232-4. [Medline].

  3. Karcioglu ZA, Mullaney PB. Diagnosis and management of iris juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus. Jan-Feb 1997;34(1):44-51. [Medline].

  4. Vendal Z, Walton D, Chen T. Glaucoma in juvenile xanthogranuloma. Semin Ophthalmol. Jul-Sep 2006;21(3):191-4. [Medline].

  5. Cambiaghi S, Restano L, Caputo R. Juvenile xanthogranuloma associated wiht neurofibromatosis 1: patients without evidence of hematologic malignancies. Pediatr Dermatol. 2004;21(2):97-101. [Medline].

  6. Cadera W, Silver MM, Burt L. Juvenile xanthogranuloma. Can J Ophthalmol. Jun 1983;18(4):169-74. [Medline].

  7. Chang MW. Update on juvenile xanthogranuloma: unusual cutaneous and systemic variants. Semin Cutan Med Surg. Sep 1999;18(3):195-205. [Medline].

  8. DeBarge LR, Chan CC, Greenberg SC, et al. Chorioretinal, iris, and ciliary body infiltration by juvenile xanthogranuloma masquerading as uveitis. Surv Ophthalmol. Jul-Aug 1994;39(1):65-71. [Medline].

  9. Harley RD, Romayananda N, Chan GH. Juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus. Jan-Feb 1982;19(1):33-9. [Medline].

  10. Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol. Mar 1997;36(3 Pt 1):355-67; quiz 368-9. [Medline].

  11. Kaur H, Cameron JD, Mohney BG. Severe astigmatic amblyopia secondary to subcutaneous juvenile xanthogranuloma of the eyelid. J AAPOS. Jun 2006;10(3):277-8. [Medline].

  12. Miszkiel KA, Sohaib SA, Rose GE, et al. Radiological and clinicopathological features of orbital xanthogranuloma. Br J Ophthalmol. Mar 2000;84(3):251-8. [Medline].

  13. Shields JA, Shields CL. Clinical spectrum of histiocytic tumors of the orbit. Trans Pa Acad Ophthalmol Otolaryngol. 1990;42:931-7. [Medline].

  14. Tanz WS, Schwartz RA, Janniger CK. Juvenile xanthogranuloma. Cutis. Oct 1994;54(4):241-5. [Medline].

  15. Weitzman S, Jaffe R. Uncommon histiocytic disorders: The non-Langerhans cell histiocytoses. Pediatr Blood Cancer. 2005;44:1-9. [Medline].

  16. Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol. Aug 1995;131(8):904-8. [Medline].

 
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