Clinical Features

In nearly half of the cases, the disease affects infants under 6 months of age. There is no gender predilection. Occurrence of JXG is reported to be 10 times more frequent in whites than in blacks. Juvenile xanthogranuloma typically presents as a single or several asymptomatic, small (1-2 cm in diameter), flat or dome-shaped, firm, shiny, yellow-red papules (Fig. 1). Rarely do the lesions exceed the size of 2-3 cm. Ulceration and satellite lesions have been described. Spontaneous regression occurs over a period of months or years, leaving small atrophic scars. Many different clinical variants have been described including eruptive, plaque-like, lichenoid, giant, and disfiguring forms. Attempts to correlate the number and size of the lesions with the risk of systemic involvement have not been helpful.

Extracutaneous involvement may occur in visceral organs, lymph nodes, soft tissue, and skeletal muscles. Eye involvement occurs in up to 10% of cases and may lead to secondary glaucoma due to hemorrhage into the anterior chamber (1-3). The association of JXG with neurofibromatosis I and chronic juvenile myelogenous leukemia is well-established. Many other perhaps coincidence associations have also been reported such as with Niemann-Pick disease and urticaria pigmentosa (4-6).

Curing Eczema Naturally

Curing Eczema Naturally

Do You Suffer From the Itching, Redness and Scaling of Chronic Eczema? If so you are not ALONE! It strikes men and women young and old! It is not just

Get My Free Ebook

Post a comment