Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement
Makaleler > Tam Makale · 2021
Yazarlar: Eda ATASEVEN, Deniz Kizmazoglu, Haldun Oniz
Pediatric Dermatology, Uluslararası, Wos, 2021, cilt 38, ss 892-894
DOI: 10.1111/pde.14634
Özet
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).