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Medientyp:
E-Artikel
Titel:
Erdheim-Chester disease: a rare non-Langerhans histiocytosis
Beteiligte:
Costa, Sofia;
Julião, Maria José;
Silva, Sónia;
Brito, Manuel João
Erschienen:
BMJ, 2021
Erschienen in:
BMJ Case Reports, 14 (2021) 9, Seite e241143
Sprache:
Englisch
DOI:
10.1136/bcr-2020-241143
ISSN:
1757-790X
Entstehung:
Anmerkungen:
Beschreibung:
A 3-year-old girl was admitted to our hospital with diabetes insipidus and a left eye proptosis. During investigation of diabetes insipidus, an extensive osteolytic mass, involving skull base and maxillo-facial bones, was revealed. Biopsy exhibited dense infiltrate of foamy histiocytes, which were positive for CD68 and CD163 and negative for CD1a and S100 confirming histopathological diagnosis of Erdheim-Chester disease. Treatment with dabrafenib was initiated, with good response and no side effects.