Erdheim-Chester disease in a child with MR imaging showing regression of marrow changes.
OBJECTIVE
Erdheim-Chester disease is a disseminated xanthogranulomatous infiltrative disease of unknown origin that generally presents in adulthood. A review of the English-language literature demonstrated that pediatric cases were extremely rare, and to our knowledge, only two cases, a 7- and 14-year-old, have been published.


METHODS
We report a case of Erdheim-Chester disease in a 10-year-old girl evaluated with MR imaging. Radiographs revealed typical bilateral, symmetric osteosclerosis of the metaphyseal regions of long bones of the upper and lower extremities.


RESULTS
A histologic examination demonstrated foamy histiocytes in bone marrow smears. Bilateral symmetric low signal intensities of both proximal tibiae and distal femurs were demonstrated on T1-weighted MR images. After oral steroid therapy for 8 months, follow-up MR imaging showed remarkable restoration of normal high signal intensity in both the tibial and femoral metaphyses.


CONCLUSIONS
To our knowledge, this may be the first case of Erdheim-Chester disease that showed normal restoration of the abnormal signal intensities in the metaphyses of long bones after steroid therapy.
Joo
Chan Uhng
CU
Department of Pediatrics, Chonbuk National University Medical School, 561-712 Jeonbuk, Korea.
Go
Yang Sim
YS
Kim
In Hwan
IH
Kim
Chul Seong
CS
Lee
Sang Yong
SY
D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D011239:Prednisolone / Q000627:therapeutic use
D005938:Glucocorticoids; D011239:Prednisolone