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.
Department of Pediatrics, Chonbuk National University Medical School, 561-712 Jeonbuk, Korea.
Chan Uhng
CU
Joo
Yang Sim
YS
Go
In Hwan
IH
Kim
Chul Seong
CS
Kim
Sang Yong
SY
Lee
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