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Bone Involvement in Erdheim-Chester Disease: Imaging Findings including Periostitis and Partial Epiphyseal Involvement¹

¹ From the Departments of Radiology (E.D., C.G., D.Z., P.A.G.) and Internal Medicine (J.H., B.W., Z.A., J.C.P.), La Pitié Salpêtrière Hospital, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France; Department of Radiology, Bicêtre Hospital, Paris, France (A.M.); and Department of Radiology, Lariboisière Hospital, Paris, France (J.D.L.). Received September 3, 2004; revision requested November 24; revision received January 24, 2005; accepted February 24; final version accepted April 11. Address correspondence to E.D. (e-mail: elisabeth.dion{at}psl.ap-hop-paris.fr).

View larger version (102K): [in a new window]   Figure 1: AP radiograph of two legs in a 52-year-old man. Bone involvement is bilateral and symmetric and consists of diaphyseal and metaphyseal heterogeneous osteosclerosis (white arrow). Shape of the tibia is tubular because of thickening of the cortex on both its endosteal and periosteal aspects. Corticomedullary margins in the diaphysis are blurred, and the marrow cavity is obliterated (black arrow).

View larger version (81K): [in a new window]   Figure 2: AP radiograph of two legs in a 36-year-old woman shows symmetric osteosclerosis of both tibiae, which also involves the proximal and distal epiphyses. A radiolucent band (arrows) separates metaphyseal and epiphyseal osteosclerosis.

View larger version (153K): [in a new window]   Figure 3a: (a) Oblique radiographs of distal femora in a 44-year-old woman show diaphyseal and metaphyseal heterogeneous osteosclerosis. Distal femora are enlarged, and periostitis is visible as a wavy contour (arrows) of the outer cortex. (b) Coronal T1-weighted spin-echo (350/15) MR image of both knees demonstrates symmetric low signal intensity of diaphyses and metaphyses. (c) Fat-suppressed T2-weighted spin-echo (2000/80) and (d) fat-suppressed gadolinium-enhanced T1-weighted spin-echo (340/15) MR images show heterogeneous signal intensity in the same areas. Epiphyses are partially involved with persistent fatty areas. Periostitis is visible as a line of high signal intensity at the outer aspect of femoral cortices (arrow). Osteoarthritis was also present and responsible for joint effusion and synovitis.

View larger version (197K): [in a new window]   Figure 3b: (a) Oblique radiographs of distal femora in a 44-year-old woman show diaphyseal and metaphyseal heterogeneous osteosclerosis. Distal femora are enlarged, and periostitis is visible as a wavy contour (arrows) of the outer cortex. (b) Coronal T1-weighted spin-echo (350/15) MR image of both knees demonstrates symmetric low signal intensity of diaphyses and metaphyses. (c) Fat-suppressed T2-weighted spin-echo (2000/80) and (d) fat-suppressed gadolinium-enhanced T1-weighted spin-echo (340/15) MR images show heterogeneous signal intensity in the same areas. Epiphyses are partially involved with persistent fatty areas. Periostitis is visible as a line of high signal intensity at the outer aspect of femoral cortices (arrow). Osteoarthritis was also present and responsible for joint effusion and synovitis.

View larger version (197K): [in a new window]   Figure 3c: (a) Oblique radiographs of distal femora in a 44-year-old woman show diaphyseal and metaphyseal heterogeneous osteosclerosis. Distal femora are enlarged, and periostitis is visible as a wavy contour (arrows) of the outer cortex. (b) Coronal T1-weighted spin-echo (350/15) MR image of both knees demonstrates symmetric low signal intensity of diaphyses and metaphyses. (c) Fat-suppressed T2-weighted spin-echo (2000/80) and (d) fat-suppressed gadolinium-enhanced T1-weighted spin-echo (340/15) MR images show heterogeneous signal intensity in the same areas. Epiphyses are partially involved with persistent fatty areas. Periostitis is visible as a line of high signal intensity at the outer aspect of femoral cortices (arrow). Osteoarthritis was also present and responsible for joint effusion and synovitis.

View larger version (208K): [in a new window]   Figure 3d: (a) Oblique radiographs of distal femora in a 44-year-old woman show diaphyseal and metaphyseal heterogeneous osteosclerosis. Distal femora are enlarged, and periostitis is visible as a wavy contour (arrows) of the outer cortex. (b) Coronal T1-weighted spin-echo (350/15) MR image of both knees demonstrates symmetric low signal intensity of diaphyses and metaphyses. (c) Fat-suppressed T2-weighted spin-echo (2000/80) and (d) fat-suppressed gadolinium-enhanced T1-weighted spin-echo (340/15) MR images show heterogeneous signal intensity in the same areas. Epiphyses are partially involved with persistent fatty areas. Periostitis is visible as a line of high signal intensity at the outer aspect of femoral cortices (arrow). Osteoarthritis was also present and responsible for joint effusion and synovitis.

View larger version (164K): [in a new window]   Figure 4a: (a) Coronal T1-weighted spin-echo (340/15) and (b) fat-saturated T2-weighted spin-echo (2000/80) MR images of two knees in a 54-year-old man. Diffuse low signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images are visible in diaphysis and metaphysis of distal femora and proximal tibiae. The fatty marrow of the subchondral bone is preserved with well-defined margins (arrows).

View larger version (144K): [in a new window]   Figure 4b: (a) Coronal T1-weighted spin-echo (340/15) and (b) fat-saturated T2-weighted spin-echo (2000/80) MR images of two knees in a 54-year-old man. Diffuse low signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images are visible in diaphysis and metaphysis of distal femora and proximal tibiae. The fatty marrow of the subchondral bone is preserved with well-defined margins (arrows).

View larger version (59K): [in a new window]   Figure 5a: (a) AP and (b) lateral radiographs and (c) coronal and (d) sagittal T1-weighted spin-echo (340/15) MR images of the left knee in a 45-year-old man. Epiphysis involvement is clearly depicted as an area of low signal intensity on the MR images. Areas of fatty marrow are preserved in the subchondral bone and metaphyses (arrows).

View larger version (64K): [in a new window]   Figure 5b: (a) AP and (b) lateral radiographs and (c) coronal and (d) sagittal T1-weighted spin-echo (340/15) MR images of the left knee in a 45-year-old man. Epiphysis involvement is clearly depicted as an area of low signal intensity on the MR images. Areas of fatty marrow are preserved in the subchondral bone and metaphyses (arrows).

View larger version (136K): [in a new window]   Figure 5c: (a) AP and (b) lateral radiographs and (c) coronal and (d) sagittal T1-weighted spin-echo (340/15) MR images of the left knee in a 45-year-old man. Epiphysis involvement is clearly depicted as an area of low signal intensity on the MR images. Areas of fatty marrow are preserved in the subchondral bone and metaphyses (arrows).

View larger version (61K): [in a new window]   Figure 5d: (a) AP and (b) lateral radiographs and (c) coronal and (d) sagittal T1-weighted spin-echo (340/15) MR images of the left knee in a 45-year-old man. Epiphysis involvement is clearly depicted as an area of low signal intensity on the MR images. Areas of fatty marrow are preserved in the subchondral bone and metaphyses (arrows).

View larger version (159K): [in a new window]   Figure 6a: MR images of the brain and face in a 48-year-old man. (a) Coronal T1-weighted spin-echo (340/12) image shows a large sinus mass (arrow) extending into orbits and associated with bone destruction. (b, c) Gadolinium-enhanced T1-weighted spin-echo (340/12) images in (b) coronal and (c) transverse planes show intense heterogeneous enhancement of the mass (arrow in b).

View larger version (173K): [in a new window]   Figure 6b: MR images of the brain and face in a 48-year-old man. (a) Coronal T1-weighted spin-echo (340/12) image shows a large sinus mass (arrow) extending into orbits and associated with bone destruction. (b, c) Gadolinium-enhanced T1-weighted spin-echo (340/12) images in (b) coronal and (c) transverse planes show intense heterogeneous enhancement of the mass (arrow in b).

View larger version (187K): [in a new window]   Figure 6c: MR images of the brain and face in a 48-year-old man. (a) Coronal T1-weighted spin-echo (340/12) image shows a large sinus mass (arrow) extending into orbits and associated with bone destruction. (b, c) Gadolinium-enhanced T1-weighted spin-echo (340/12) images in (b) coronal and (c) transverse planes show intense heterogeneous enhancement of the mass (arrow in b).

View larger version (63K): [in a new window]   Figure 7a: (a) AP radiograph of the distal femur and knee, (b) transverse CT image, and (c) fat-suppressed T2-weighted spin-echo (2000/40) MR image obtained through the distal femur in a 63-year-old man. Heterogeneous osteosclerosis of the cancellous bone is clearly depicted on b (arrow). Heterogeneous high signal intensity is visible in the femur epiphysis, while signal intensity of the patella is normal on c.

View larger version (55K): [in a new window]   Figure 7b: (a) AP radiograph of the distal femur and knee, (b) transverse CT image, and (c) fat-suppressed T2-weighted spin-echo (2000/40) MR image obtained through the distal femur in a 63-year-old man. Heterogeneous osteosclerosis of the cancellous bone is clearly depicted on b (arrow). Heterogeneous high signal intensity is visible in the femur epiphysis, while signal intensity of the patella is normal on c.

View larger version (172K): [in a new window]   Figure 7c: (a) AP radiograph of the distal femur and knee, (b) transverse CT image, and (c) fat-suppressed T2-weighted spin-echo (2000/40) MR image obtained through the distal femur in a 63-year-old man. Heterogeneous osteosclerosis of the cancellous bone is clearly depicted on b (arrow). Heterogeneous high signal intensity is visible in the femur epiphysis, while signal intensity of the patella is normal on c.