Dermatologic Manifestations of Juvenile Xanthogranuloma Treatment & Management

Updated: Oct 17, 2019
  • Author: Sally H Monahan, MD; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Anticipatory care, with patient reassurance, is appropriate because of the self-limiting benign nature of juvenile xanthogranulomas (JXGs). Ocular and systemic lesions may respond to steroids or radiotherapy. Rare cases of severe systemic JXG have required single or multiagent chemotherapy regimens.

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Surgical Care

Lesions may be excised for diagnostic and cosmetic reasons. Ocular and systemic lesion excision usually is curative. A 2008 case report describes a newborn with multiple cutaneous and hepatic juvenile xanthogranulomas (JXGs) requiring liver transplantation secondary to cholestasis and portal hypertension. [28]

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Consultations

Routine referral of patients with a juvenile xanthogranuloma (JXG) is unnecessary.

Patients younger than 2 years with multiple skin lesions comprise 92% of associated cases of ocular involvement. Refer these patients to an ophthalmologist, and continue screening every 6 months through the second year of life. Some have also recommended an ophthalmologist referral for those with periocular JXGs.

When presented with children diagnosed with both JXG and type 1 neurofibromatosis (NF1), physicians should be on alert for possible juvenile myelomonocytic leukemia (JMML). Whether screening for JMML is necessary remains controversial, and little evidence supports performing hematological panels for detecting malignancy. Instead, physicians should look first for clinical signs of JMML in patients with NF1 and JXG, particularly hepatosplenomegaly, lymphadenopathy, and/or pallor.

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Long-Term Monitoring

Follow-up visits may be scheduled at regular intervals for reassurance and to monitor associated complications. For patients with juvenile xanthogranulomas (JXGs) and type 1 neurofibromatosis (NF1), physicians should watch for signs and symptoms of juvenile myelomonocytic leukemia (JMML). [29]

Patients with ocular involvement should be checked regularly by an ophthalmologist to prevent rare complications, such as glaucoma. [30]

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