Juvenile Xanthogranuloma

Excerpt

Juvenile xanthogranuloma (JXG) is a relatively common entity and is the most common form of non-Langerhans cell histiocytic disorder of childhood., It is estimated that in 75% of cases, lesions appear during the first year of life, with >15-20% of patients having lesions at birth. JXG is rare in adults, with a peak incidence in the late twenties to thirties. Rarely, reports exist of lesions arising in the elderly. The majority of adult patients have solitary lesions. The true incidence, however, may be underestimated, as many lesions, especially those which are solitary and small (up to 90% of all patients), may go unrecognized. Typically, the clinical presentation consists of solitary or multiple yellow-orange-brown firm papules or nodules. The most common locations are the face, neck, and upper torso. Oral lesions are rare and often occur as a yellow nodule on the lateral aspects of the tongue. Oral lesions can also arise on the gingival, buccal mucosa, and midline hard palate and may ulcerate and bleed. Lesions of the oral mucosa may appear verrucous, umbilicated, pedunculated, or fibroma-like. Cutaneous lesions are usually asymptomatic, and most lesions spontaneously involute over the course of several years. Although occurring rarely, ocular involvement is the most common extracutaneous site involved, followed by the lungs. Ocular JXG is nearly always unilateral and develops in less than 0.5% of patients. Approximately 40% of patients with ocular JXG, however, have multiple cutaneous lesions at the time of diagnosis. Ocular disease usually occurs prior to 2 years of age and commonly affects the iris. Hyphema and glaucoma are serious complications that can result in blindness and necessitate early referral to an ophthalmologist for evaluation and possible treatment. Other bone, visceral and or CNS involvement (e.g. diabetes insipidus) is unusual. Disease-related deaths are rare.

As opposed to other xanthomatous diseases, juvenile xanthogranulomas are not associated with metabolic or lipid disorders.