Xanthogranuloma, Juvenile

From WikiGenetics

1 Name of Condition
2 Definition
3 Description, Signs and Symptoms
4 Inheritance
5 Demographics
6 Diagnosis
7 Treatment and Management
8 Prognosis
9 Resources
10 References

[edit] Name of Condition

Juvenile Xanthogranuloma, also known as JXG.

[edit] Definition

Juvenile Xanthogranuloma is a rare benign genetic disorder. Most patients with JXG only have skin disease and its first manifestations are little orange, sometimes reddish-brown bumps that are called papules (little bumps on the skin). The symptoms frequently regress on their own, although in some cases papules may occur in other parts of the body.

[edit] Description, Signs and Symptoms

Orange or reddish-brown papules form on the skin, often on the face, forehead, and near the eyes. Sometimes they form beneath the skin, in the eye, on the brain or spinal cord, or on internal organs such as the liver, spleen, or lungs. These lesions usually require some form of treatment to avoid complications.

[edit] Inheritance

[edit] Demographics

Onset occurs in childhood, at an average age of 22 months. About 10% of cases have their onset in adulthood, which is known as Xanthogranuloma. Most cases that have early onset resolve by age 5.

Xanthogranuloma in both children and adults is most frequently found in Caucasians.

[edit] Diagnosis

After the first lesions occur in childhood, a biopsy of a lesion can diagnose JXG.

[edit] Treatment and Management

There is no cure for JXG, but symptoms sometimes disappear on their own. When the condition is present on skin only, there is usually no treatment administered. If the condition is complicated by multiple lesions present throughout the body, surgery, chemotherapy, or radiation therapy may be considered.