Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement
Articles > Article ยท 2021
Authors: Eda ATASEVEN, Deniz Kizmazoglu, Haldun Oniz
Pediatric Dermatology, International, WoS, 2021, vol 38, pp 892-894
DOI: 10.1111/pde.14634
Summary
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).