Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

Juvenile xanthogranuloma (JXG) is a common form of non-Langerhans cell histiocytosis, which usually presents with spontaneously regressing skin lesions. Systemic involvement is rare and mostly seen in patients with multiple skin nodules. It can spontaneously regress, but sometimes systemic involvement can cause life-threatening symptoms and can be fatal. Herein, we report a case of congenital systemic JXG with multiple skin nodules, soft tissue and pulmonary involvement. She was successfully treated with chemotherapy according to Langerhans cell histiocytosis treatment protocol IV of the Histiocyte Society (LCH-IV).

Automated summary

Juvenile xanthogranuloma (JXG) is a common form of non-Langerhans cell histiocytosis that usually presents with spontaneously regressing skin lesions. However, systemic involvement can be life-threatening and may require chemotherapy following the treatment protocol for Langerhans cell histiocytosis.