Juvenile xanthogranuloma

Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis (see this term) characterized by the occurrence of one or more reddish or yellowish self-limiting and benign papules or nodules of several millimeters in diameter, usually appearing on the head and neck (but sometimes on the extremities and trunk) during the first year of life (or rarely in adulthood) and usually regressing spontaneously. Extracutaneous involvement has also been reported, involving most commonly the eye (uveal tract) but with other locations including the central nervous system, lung, liver, bones and endocrine glands, and may be associated with considerable morbidity.

Granuloma

A compact, organized collection of mature mononuclear phagocytes, which may be but is not necessarily accompanied by accessory features such as necrosis.


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PMID (PMCID)
28871936
FEMALE
Xanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile.
Nockowski P, Wozniak Z, Reich A, Maj J.
Acta Dermatovenerol Croat. 2017;25(2):167-169.
Aside from hyperlipemic and normolipemic xanthomas, the differential diagnosis of NXG includes multifocal necrobiosis lipoidica, granuloma annulare, foreign-body granuloma, juvenile xanthogranuloma, rheumatoid nodules, and amyloidosis (4).
28871936
FEMALE
Xanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile.
Nockowski P, Wozniak Z, Reich A, Maj J.
Acta Dermatovenerol Croat. 2017;25(2):167-169.
Aside from hyperlipemic and normolipemic xanthomas, the differential diagnosis of NXG includes multifocal necrobiosis lipoidica, granuloma annulare, foreign-body granuloma, juvenile xanthogranuloma, rheumatoid nodules, and amyloidosis (4).
27282913
MALE Child
Conjunctival fibrous histiocytoma in an 8-year-old boy.
Knapp AN, Samara WA, Shields CL, Shields JA, Eagle RC Jr.
J AAPOS. 2016;20(4):368-70.
This rare tumor can resemble several conditions, including scleritis/episcleritis, inflamed pterygium, juvenile xanthogranuloma, foreign body granuloma, solitary fibrous tumor, amelanotic melanoma, and squamous cell carcinoma.
25490241
MALE
Presumed ocular juvenile xanthogranuloma and biopsy-proven cutaneous mastocytosis occurring sequentially in a young boy.
Djougarian A, Kodsi S, Naysan J, Kristal L, Marr B.
J Pediatr Ophthalmol Strabismus. 2014;51 Online:e89-91.
Ocular lesions can occur in juvenile xanthogranuloma, most commonly presenting as an iris granuloma.
17123317
MALE Infant
Managing isolated subglottic juvenile xanthogranuloma without tracheostomy: case report and review of literature.
Somorai M, Goldstein NA, Alexis R, Giusti RJ.
Pediatr Pulmonol. 2007;42(2):181-5.
Juvenile Xanthogranuloma (JXG) is a dendritic cell related histiocytic disorder which usually presents in the first year of life as a solitary cutaneous granuloma.
16121556
FEMALE
Chorioretinal involvement and vitreous hemorrhage in a patient with juvenile xanthogranuloma.
Berrocal AM, Davis JL.
J Pediatr Ophthalmol Strabismus. 2005;42(4):241-2.
Juvenile xanthogranuloma may be associated with posterior manifestations including optic nerve and choroidal granulomas, panuveitis, and vitreous hemorrhage in the absence of the classic anterior segment manifestations.
15492746
FEMALE Infant
Cavernous hemangioma of the iris in an infant.
Woo SJ, Kim CJ, Yu YS.
J AAPOS. 2004;8(5):499-501.
Juvenile xanthogranuloma, malignant melanoma with proliferation of vessels or hemorrhages in the region of cystic degeneration, and inflammatory granulomas are frequently confused with the true hemangiomas.