Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

ATASEVEN E., Kizmazoglu D., Oniz H.

PEDIATRIC DERMATOLOGY, cilt.38, sa.4, ss.892-894, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 38 Sayı: 4
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1111/pde.14634
  • Dergi Adı: PEDIATRIC DERMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.892-894
  • Anahtar Kelimeler: congenital juvenile xanthogranuloma, lung metastases, systemic juvenile xanthogranuloma, DISEASE, TUMOR
  • Dokuz Eylül Üniversitesi Adresli: Evet

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).