A case report of pediatric systemic juvenile xanthogranuloma

Abstract

Background: Juvenile xanthogranuloma (JXG) is a rare disorder that belongs to the broad group of non-Langerhans cell histiocytosis. It is characterized by one or more nodules with predilection sites on the head, neck, and trunk, and lesions that may be several millimeters in diameter. These are reddish or yellowish benign papules or nodules that usually resolve spontaneously. The involvement of organs other than the skin is termed systemic juvenile xanthogranuloma (SJXG). The eye is the most frequent extracutaneous location of the JXG.

Case presentation: We report a case of SJXG in a male child, with onset in the second month of life. He presented with several nodules, approximately 5 mm in diameter and tan-orange in color, located on the head, face, and trunk. The nodules enlarged to 10 mm in diameter, and new lesions were found in the right eye, which resulted in spontaneous hyphema and secondary glaucoma without treatment. The pathological findings suggested that the nodule was of histiocytic origin, and immunohistochemical analysis resulted in the diagnosis of JXG. Chemotherapy based on the Langerhans cell histiocytosis (LCH) regimen resulted in a good prognosis.

Conclusion: SJXG has low morbidity, but is unpredictable, and rare and self-limited. Treatment is required for patients with extracutaneous involvement, who may have increased morbidity. The LCH-Ⅲ protocol of the International Histiocyte Society is the most commonly used and effective chemotherapy regimen.

Keywords

juvenile xanthogranuloma / systemic juvenile xanthogranuloma / treatment

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