Xanthogranuloma

Key Points
Rare form of histiocytosis, a condition in which there are excessive levels of histiocyte cells in the body
Cause is unknown
Typically appears in infants and children, though it may occur in adults
Initially consists of small, pink bumps in the affected area, which may become yellow and scaly as the condition progresses

Juvenile xanthogranuloma is a rare form of histiocytosis, a condition in which there excessive amounts of histiocytes (an immune system cell) in the body. It initially consists of small, pink bumps, which may become yellow and/or scaly as the condition progresses. Lesions may also increase in size, up to 2 cm. Lesions typically form on the trunk and upper extremities, but may appear anywhere on the body, in the eye, or in the internal organs.

The cause of juvenile xanthogranuloma is unknown, but it has been shown to affect primarily infants and small children, with males being more likely to develop the condition. Adult cases are much more infrequent, and the condition is more common in caucasians than other ethnic groups. The condition is difficult to distinguish from other disorders, including benign cephalic histiocytosis, generalized eruptive histiocytosis, and Papular xanthogranuloma. It is thought that these disorders may all be the same condition.

Differential Diagnosis (Other conditions with similar appearance)
Glaucoma
Pigmented Lesions of the Eyelid
Retinoblastoma
Retinopathy of Prematurity
Hyphema
Uveitis, Anterior, Childhood

Diagnosis
Key Points
Initial diagnosis based on appearance of the affected area
Skin biopsy will be performed to confirm diagnosis and rule out other conditions

Juvenile xanthogranuloma is initially diagnosed based on the appearance of the affected area. A skin biopsy will confirm the diagnosis and rule out other conditions.

Treatment
Lesions are typically harmless, unless they affect the eyes
Removal generally unnecessary and lesions will spontaneously resolve over a period of 2-3 years without scarring
*Surgical removal is possible, but will leave scarring

Treatment of juvenile xanthogranuloma is typically not required, as lesions are harmless unless they affect the eyes. This make removal generally unnecessary, and lesions will typically resolve over a period of 2-3 years without scarring. If required, surgical removal is possible, but will result in scarring of the affected area.