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Juvenile xanthogranulomas: Examining single, multiple, and extracutaneous presentations.
Naomi So1, Regina Liu1, Marcia Hogeling2
1David Geffen School of Medicine at UCLA, Los Angeles, California.
Pediatric Dermatology
|May 30, 2020
Summary
Juvenile xanthogranuloma (JXG) is a skin condition typically appearing in infants. While often self-resolving, multiple or extracutaneous JXG lesions may require specialist evaluation.
Area of Science:
- Dermatology
- Pediatric Histiocytosis
Background:
- Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis.
- Characterized by yellowish skin papules, JXG commonly affects infants and young children.
- While typically solitary and self-regressing, extracutaneous JXG lesions can occur.
Purpose of the Study:
- Review literature on single, multiple, and visceral JXG.
- Provide recommendations for monitoring and diagnostic work-up.
- Clarify JXG presentations, including associations with NF1 and JMML.
Main Methods:
- Conducted a PubMed literature review.
- Utilized selective search terms for various JXG lesion types and associated conditions.
- Focused on single, multiple, ocular, visceral JXG, and NF1/JMML.
Main Results:
- JXG is usually self-limited with cutaneous, singular lesions.
- Rarely, JXG can present as multiple or extracutaneous lesions.
- Screening and specialist referral may be necessary for complex cases.
Conclusions:
- JXG commonly presents as single, multiple, or extracutaneous lesions.
- JXG can be associated with neurofibromatosis type 1 (NF1) and juvenile myelomonocytic leukemia (JMML).
- Case-by-case evaluation by a dermatologist and specialist referral are recommended.
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