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Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement
Author(s) -
Ataseven Eda,
Kizmazoglu Deniz,
Oniz Haldun
Publication year - 2021
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.14634
Subject(s) - medicine , juvenile xanthogranuloma , histiocyte , langerhans cell histiocytosis , histiocytosis , pathology , juvenile , systemic therapy , chemotherapy , langerhans cell , systemic disease , dermatology , surgery , immunology , immunopathology , disease , cancer , antigen , biology , breast cancer , genetics
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).