Introduction:
Erdheim-Chester disease (ECD) is a rare multisystemic disease of undetermined origin, clinically characterized by multi organ involvement, affecting primarily middle-aged adults (1) preponderantly males. Histologically ECD is characterized by the presence of foamy-macrophages in a fibrotic inflammatory stroma. Histiocytes test positive for CD 68 and CD163 but negative for CD1a. Molecular testing for BRAF and MAPK-ERK pathway mutations has opened the doors for targeted therapies.
The clinical spectrum of ECD is wide and includes frequently bone lesions, cardiovascular manifestations, retroperitoneum and kidney involvement, endocrine manifestations, central nervous system, respiratory involvement and dermatologic lesions. Genital organs and digestif tract involvement are rare (2).
We herein report the case of a patient illustrating the muti-organ manifestations of ECD with rare manifestations such as breast and liver lesions.