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MRI Findings in Deep and Generalized Morphea (Localized Scleroderma)

¹ Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str. 3, Tübingen 72076, Germany.
² Department of Dermatology, Eberhard-Karls-University, Tübingen 72070, Germany.
³ Department of Pediatrics, Eberhard-Karls-University, Tübingen 72076, Germany.
⁴ Department of Pathology, Eberhard-Karls-University, Tübingen 72076, Germany.
⁵ Present address: Russel H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287.

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —9-year-old boy with deep pansclerotic disabling morphea. Photomicrographs show dermis is increased in thickness and composed of broad sclerotic collagen bundles. Collagen has replaced fat around sweat glands (arrowhead, A) and extends into subcutis. Eccrine glands are situated at relatively high level in dermis as a result of collagen deposited below them. Scattered lymphocytes are located around blood vessels (long black arrow, A). Note dense collagenization of dermis and extension of fibrous tissue (short black arrow, A) into subcutaneous fat (white arrow) (A) (H and E, x50) and musculature (long arrow, B) are shown in this patient with deep morphea. Below sclerotic subcutis, collagen bundles (short arrows, B) are located between skeletal muscle fibers (long arrow, B) in deep morphea (B) (Van Gieson stain, x100).

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —9-year-old boy with deep pansclerotic disabling morphea. Photomicrographs show dermis is increased in thickness and composed of broad sclerotic collagen bundles. Collagen has replaced fat around sweat glands (arrowhead, A) and extends into subcutis. Eccrine glands are situated at relatively high level in dermis as a result of collagen deposited below them. Scattered lymphocytes are located around blood vessels (long black arrow, A). Note dense collagenization of dermis and extension of fibrous tissue (short black arrow, A) into subcutaneous fat (white arrow) (A) (H and E, x50) and musculature (long arrow, B) are shown in this patient with deep morphea. Below sclerotic subcutis, collagen bundles (short arrows, B) are located between skeletal muscle fibers (long arrow, B) in deep morphea (B) (Van Gieson stain, x100).

View larger version (98K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —43-year-old man with deep morphea. Note increased signal on axial STIR (TR/TE, 7,763/70; inversion time, 150 milliseconds) image along posterior part of left forearm (arrow) due to infiltration of dermis, subcutaneous tissue, and part of muscular fascia.

View larger version (116K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —43-year-old man with deep morphea. Note corresponding moderate enhancement (arrow) in involved dermis and subcutis on T1-weighted fat-suppressed spin-echo (TR/TE, 655/17) image acquired after IV administration of gadopentetate dimeglumine.

View larger version (103K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C —43-year-old man with deep morphea. Effacement of normal high signal of subcutaneous fatty tissue and skin thickening caused by collagen infiltration show hypointense signal (arrow) on T1-weighted unenhanced spin-echo (TR/TE, 561/12) image. There is no relevant binding down of skin over involved forearm in this patient with short history of morphea.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —9-year-old boy with deep pansclerotic disabling morphea (same patient as in Figure 1A, 1B). Axial STIR (TR/TE, 6,210/35; inversion time, 150 milliseconds) image shows mild signal hyperintensity of thickened skin in right calf (short arrow). In addition, high signal intensity is seen in posterior muscle compartment (arrowhead) and tibial bone marrow (long arrow).

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —9-year-old boy with deep pansclerotic disabling morphea (same patient as in Figure 1A, 1B). Axial T1-weighted spin-echo (TR/TE, 470/17) image also shows thickening of skin and reticular infiltration of subcutaneous fatty tissue over tibial bone due to collagen (arrows). There was only mild enhancement of involved musculature on T1-weighted fat-suppressed spin-echo (TR/TE, 646/17) image acquired after IV administration of gadopentetate dimeglumine (not shown).

View larger version (59K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C —9-year-old boy with deep pansclerotic disabling morphea (same patient as in Figure 1A, 1B). Coronal STIR (TR/TE, 7,640/58; inversion time, 150 milliseconds) image of right tibia illustrates diffuse bone marrow infiltration by highly cellular fibrous tissue with increased signal.

View larger version (81K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D —9-year-old boy with deep pansclerotic disabling morphea (same patient as in Figure 1A, 1B). Furthermore, left hindfoot shows diffuse bone marrow infiltration on STIR (TR/TE, 8,910/58; inversion time, 150 milliseconds) image (short arrow). T2 and STIR signals are more intense than expected in patients with bone marrow edema, and distribution does not follow expected pattern for edema. Longer arrow indicates bone marrow edema in contralateral heel.

View larger version (61K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3E —9-year-old boy with deep pansclerotic disabling morphea (same patient as in Figure 1A, 1B). After IV administration of gadolinium, moderate to intense enhancement is seen in involved bone marrow of femoral (not shown) and tibial bone (small arrow) as well as in left heel bone (large arrow).

View larger version (82K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —17-year-old girl with generalized morphea. Jaccoud-like deformity of right hand is seen on radiograph. Note decreased periarticular X-ray absorption due to osteopenia. Unlike patients presenting with systemic sclerosis, in patients with morphea acroosteolysis is unusual. Note also flexion contracture of right hand.

View larger version (103K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —17-year-old girl with generalized morphea. Axial T1-weighted fat-suppressed spin-echo (TR/TE, 620/17) gadolinium-enhanced image obtained at level of right wrist joint reveals synovitis (short arrow) of distal radioulnar joint and mild tendon sheath synovitis of flexor (long arrow) and especially extensor muscles, including all compartments.

View larger version (96K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4C —17-year-old girl with generalized morphea. Coronal T1-weighted fat-suppressed spin-echo (TR/TE, 646/17) gadolinium-enhanced image shows mild thickening and synovial enhancement of flexor sheaths (arrows). There were no erosions in hand joints (not shown).

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5 —43-year-old man with deep morphea (same patient as in Fig. 2A, 2B, 2C). Note tendon sheath synovitis including all compartments of forearm musculature (short and long arrows) as shown on this axial T1-weighted fat-suppressed spin-echo (TR/TE, 512/11) image. Note intense gadolinium enhancement, especially in tendon sheaths of flexor digitorum muscles (long arrow).

View larger version (67K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —9-year-old boy with deep pansclerotic disabling morphea who presented with bone involvement (same patient as in Fig. 1A, 1B). Coronal T1-weighted fat-suppressed spin-echo (TR/TE, 892/11) gadolinium-enhanced image shows medullar infiltration of right tibial diaphysis (arrows) with inhomogeneous moderate enhancement before therapy.

View larger version (48K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —9-year-old boy with deep pansclerotic disabling morphea who presented with bone involvement (same patient as in Fig. 1A, 1B). Coronal T1-weighted fat-suppressed spin-echo (TR/TE, 531/11) gadolinium-enhanced image shows almost entire resolution of this infiltrate (arrows) after high-dose chemotherapy and subsequent autologous hematopoietic stem cell transplantation.

View larger version (120K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A —77-year-old woman with generalized morphea. Axial T1-weighted fat-suppressed spin-echo (TR/TE, 670/11) gadolinium-enhanced image shows fascial thickening and curvilinear enhancement (arrow) in left thigh and involvement of biceps femoris muscle.

View larger version (111K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B —77-year-old woman with generalized morphea. Axial T1-weighted fat-suppressed 2D gradient-echo (TR/TE, 130/4.13) gadolinium-enhanced images show linear thickening and reticulation of subcutaneous fatty tissue (arrows), fascial thickening (arrows, B and long arrow, C), and gadolinium enhancement in pelvic region. Lower arrowhead in C indicates collagen septal thickening.

View larger version (82K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7C —77-year-old woman with generalized morphea. Axial T1-weighted fat-suppressed 2D gradient-echo (TR/TE, 130/4.13) gadolinium-enhanced images show linear thickening and reticulation of subcutaneous fatty tissue (arrows), fascial thickening (arrows, B and long arrow, C), and gadolinium enhancement in pelvic region. Lower arrowhead in C indicates collagen septal thickening.

View larger version (102K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7D —77-year-old woman with generalized morphea. Axial T1-weighted fat-suppressed 2D gradient-echo (TR/TE, 130/4.76) enhanced image shows strong gadolinium enhancement by involvement of muscular fasciae at torso (arrows). Fascial involvement follows distribution of skin changes in patients with morphea, unlike other diseases that mimic morphea.

View larger version (107K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7E —77-year-old woman with generalized morphea. Axial T1-weighted fat-suppressed spin-echo (TR/TE, 708/11) gadolinium-enhanced axial image of left knee joint (arrow). Note also fascial thickening over biceps femoris muscle (arrowhead).

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8A —71-year-old woman with deep morphea. Axial STIR (TR/TE, 7,070/70; inversion time, 150 milliseconds) image of right thigh shows increased signal intensity in fascia of biceps femoris muscle and adductor muscles (arrows).

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8B —71-year-old woman with deep morphea. On T1-weighted unenhanced spin-echo sequence, these changes are not that obvious.

View larger version (103K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8C —71-year-old woman with deep morphea. However, axial T1-weighted fat-suppressed spin-echo (TR/TE, 6,708/10) gadolinium-enhanced image shows strong fascial enhancement resembling eosinophilic fasciitis (arrows). In this particular case, eosinophilic infiltration could not be seen at biopsy, and there was no peripheral eosinophilia.

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9 —60-year-old woman with eosinophilic fasciitis (Shulman's syndrome). Note generalized thickening of skin and intense fascial enhancement on axial T1-weighted fat-suppressed spin-echo (TR/TE, 670/10) gadolinium-enhanced image, corresponding to locations of T2 signal abnormalities (not shown). Fascial thickening (arrows) is leading MRI feature in this case. Note resemblance to findings in Figure 7D. Muscular involvement, if any, in this disorder is located along superficial and deep fascial layers and superficial muscle fibers adjacent to fascia. There is usually no synovial thickening or enhancement and no tenosynovial abnormality. Bone signal abnormalities do not belong to typical imaging features of this disorder.

View larger version (49K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10A —67-year-old man with Shulman's syndrome who presented clinically with generalized puckered skin changes and functional disability due to skin tightness. Whole-body STIR image (TR/TE, 7,960/87; inversion time, 150 milliseconds), acquired before institution of steroid therapy in this patient with eosinophilic fasciitis, shows generalized thickening and increased signal of all muscular fasciae. Note also increased signal of subcutaneous tissue.

View larger version (48K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10B —67-year-old man with Shulman's syndrome who presented clinically with generalized puckered skin changes and functional disability due to skin tightness. Whole-body coronal STIR image (TR/TE, 7,960/87; inversion time, 150 milliseconds) 12 months later shows entire resolution of subcutaneous and fascial thickening and accompanying signal abnormalities. Signal abnormalities in muscle fasciae and subcutaneous tissue parallel those on T1-weighted fat-suppressed gadolinium-enhanced images (not shown).

View larger version (95K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11A —17-year-old girl with juvenile dermatomyositis. Despite discrete cutaneous lesions (clinically corresponding to specific skin rash) over pelvic region, leading MRI finding in this patient is abnormally increased T2 signal (arrows, A) in musculature as shown on these axial STIR (TR/TE, 7,390/87; inversion time, 150 milliseconds) (A) and coronal STIR (TR/TE, 9,760/87; inversion time, 150 milliseconds) (B) images. Short arrow in A indicates increased signal in psoas and iliacus muscles due to myositis. Long arrow therefore indicates only moderate involvement of gluteus medius muscle. Arrows in B indicate signal enhancement in upper girdle musculature, thigh, and calf. Similar findings are seen in patients with polymyositis. Contrary to morphea, polymyositis and dermatomyositis have more symmetric distribution, initially involving proximal lower limb girdle and progressing to involve proximal upper limb girdle, neck flexors, and pharyngeal muscles. Patients with juvenile dermatomyositis develop calcinosis later in course of disease (not shown). In patients with morphea, muscular abnormalities are confined only to muscle groups lying below typical skin lesions.

View larger version (44K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11B —17-year-old girl with juvenile dermatomyositis. Despite discrete cutaneous lesions (clinically corresponding to specific skin rash) over pelvic region, leading MRI finding in this patient is abnormally increased T2 signal (arrows, A) in musculature as shown on these axial STIR (TR/TE, 7,390/87; inversion time, 150 milliseconds) (A) and coronal STIR (TR/TE, 9,760/87; inversion time, 150 milliseconds) (B) images. Short arrow in A indicates increased signal in psoas and iliacus muscles due to myositis. Long arrow therefore indicates only moderate involvement of gluteus medius muscle. Arrows in B indicate signal enhancement in upper girdle musculature, thigh, and calf. Similar findings are seen in patients with polymyositis. Contrary to morphea, polymyositis and dermatomyositis have more symmetric distribution, initially involving proximal lower limb girdle and progressing to involve proximal upper limb girdle, neck flexors, and pharyngeal muscles. Patients with juvenile dermatomyositis develop calcinosis later in course of disease (not shown). In patients with morphea, muscular abnormalities are confined only to muscle groups lying below typical skin lesions.

View larger version (138K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12 —59-year-old man with graft-versus-host disease (GVHD) after allogeneic stem cell transplantation for acute myelogenous leukemia. Skin thickening and induration (arrows) were obvious in this patient; however, note also fascial thickening and markedly increased enhancement in thigh musculature as shown on this axial T1-weighted fat-suppressed spin-echo (TR/TE, 691/13) gadolinium-enhanced image. Note striking resemblance to Figure 7A, representing morphea. MRI findings are almost similar in patients with GVHD and morphea; therefore, differentiation by means of imaging alone is not possible. Nevertheless, clinical setting is usually different, and both disorders have low prevalence.

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