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Juvenile xanthogranuloma in Adult
See also in: External and Internal Eye,Hair and Scalp
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Juvenile xanthogranuloma in Adult

See also in: External and Internal Eye,Hair and Scalp
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Contributors: Tyler Werbel, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Juvenile xanthogranulomas (JXG) are benign, spontaneously regressing histiocytic tumors of unknown pathogenesis. The incidence is unknown, and there may be a slight male preponderance.

JXGs are usually solitary and most often appear at birth or within the first year of life. However, their number and size may increase over the first 18 months of life. Lesions usually regress over 3-6 years, leaving behind persistent pigmentary change or atrophy.

Systemic involvement is rare, with the most common site of extracutaneous involvement being the eye (0.3%-0.5%). Eye involvement is more common in patients with multiple JXGs. Many internal organs can potentially be involved, including the central nervous system, liver, lungs, muscles, oropharynx, and spleen. Cutaneous lesions are absent in one-half of cases with systemic involvement.

JXGs have been associated with neurofibromatosis type 1 (NF1), particularly in patients younger than 2 years of age. Additionally, a triple association between JXGs, NF1, and juvenile myelomonocytic leukemia has been observed in several case reports. In adults, the condition lasts longer and is more frequently associated with permanent changes in pigment and atrophy after spontaneous resolution.

Codes

ICD10CM:
D76.3 – Other histiocytosis syndromes

SNOMEDCT:
400031009 – Juvenile xanthogranuloma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The primary differential of red-to-yellow papules on a young child is JXG, Spitz nevus, and mastocytoma.
For ocular lesions, also consider:

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 07/26/2018
Last Updated: 09/04/2018
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Juvenile xanthogranuloma in Adult
See also in: External and Internal Eye,Hair and Scalp
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View all Images (7)
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Juvenile xanthogranuloma : Cheek, Chin, Eyelids, Forehead, Iris nodules and spots, Nose, Orange color, Scattered few, Smooth nodule, Smooth papule
Clinical image of Juvenile xanthogranuloma
A dome-shaped, orange-red papule on the scalp.
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