JUVENILE XANTHOGRANULOMA

By Gary M. White, MD

Large juvenile xanthogranulomas Courtesy O. Dale Collins, MD


The juvenile xanthogranuloma (JXG) is a benign, generally asymptomatic proliferation of non-Langerhans histiocytic cells. It usually presents as a yellowish papulonodule in a child.

Clinical

Juvenile xanthogranuloma typically presents as a solitary lesion in a young child usually under 1 year of age, but it can be congenital and multiple lesions may occur. It is most common on the head and neck. When extra-cutaneous, the eye is the most common site.

Unusual variants include segmental (Pediatr Dermatol. 2013 Jul 25) and the symmetrical giant facial plaque variant. Solitary lesions may occur in adults and have been called SEX (solitary eruptive xanthomas--see photos below). Lesions in infants may occasionally ulcerate, e.g., in the groin area.

Multiple JXGs are common in children younger than 2 years with NF1. However, these are transient and spontaneously disappear before a child reaches 5 years in most cases. A comparative retrospective study did not find an increased risk for malignancy associated with JXG in children with NF-1 [JAAAD 2017;76;1084].

Systemic

Rarely, JXG may occur in extracutanous locations. These lesions are benign in nature, but can cause problems due to pressure effects or misdiagnosis as malignant entities. In one study [J Pediatr. 1996; 129: 227–237], the most common sites of extracutaneous involvement were subcutaneous soft tissue (in 33.3% of the cases), central nervous system (22.2%), liver/spleen (22.25%), lung (16.6%), eye/orbit (11.1%), and oropharynx and muscle (11.1% each).

For a discussion of the differential diagnosis of cases with multiple lesions of non-Langerhans cell Histiocyte origin, see progressive nodular histiocytosis.

Treatment

No treatment is needed however a biopsy is often done to confirm the diagnosis. Spontaneous resolution may occur over months to years without a scar. Surgical excision of course may be done and recurrence is rare. Any patients with multiple lesions (e.g. > 3) or eye symptoms should be evaluated by an ophthalmologist. IL triamcinolone has been reported as a successful treatment.

Additional Pictures

Multiple juvenile xanthogranulomas Juvenile xanthogranuloma

juvenile xanthogranuloma juvenile xanthogranuloma

JXGs in association with CALMs in a child.
Juvenile xanthogranuloma in an infant with cafe au lait macules and neurofibromatosis Cafe au lait macules in a child with neurofibromatosis and a juvenile xanthogranuloma

Solitary JXG on the forehead of an adult.
Solitary juvenile xanthogranuloma in an adult

Another solitary JXG on a young adult.
Solitary juvenile xanthogranuloma in an adult

References

Symmetric giant fascial plaques Acta Derm Venereol 2014; 94: 465–466

A segmental arrangement of JXG since birth. Pediatr Dermatol. 2014 Sep-Oct;31(5):615-7.

RegionalDerm

Homepage | FAQs | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.