Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

Eda Ataseven; Deniz Kizmazoglu; Haldun Oniz

doi:10.1111/pde.14634

Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

Pediatr Dermatol. 2021 Jul;38(4):892-894. doi: 10.1111/pde.14634. Epub 2021 May 17.

Authors

Eda Ataseven¹, Deniz Kizmazoglu², Haldun Oniz²

Affiliations

¹ Division of Pediatric Hematology and Oncology, Department of Pediatrics, Ege University School of Medicine, Izmir, Turkey.
² Division of Pediatric Hematology and Oncology, Department of Pediatrics, Izmir Tepecik Training and Research Hospital, Izmir, Turkey.

PMID: 33998710
DOI: 10.1111/pde.14634

Abstract

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).

Keywords: congenital juvenile xanthogranuloma; lung metastases; systemic juvenile xanthogranuloma.

Publication types

Case Reports

MeSH terms

Female
Histiocytosis, Langerhans-Cell* / diagnosis
Histiocytosis, Langerhans-Cell* / drug therapy
Histiocytosis, Non-Langerhans-Cell*
Humans
Skin Neoplasms*
Xanthogranuloma, Juvenile* / diagnosis
Xanthogranuloma, Juvenile* / drug therapy