DOI:10.2214/AJR.07.2163
AJR 2008; 190:32-39
© American Roentgen Ray Society
MRI Findings in Deep and Generalized Morphea (Localized Scleroderma)
Marius Horger1,
Gerhard Fierlbeck2,
Jasmin Kuemmerle-Deschner3,
Nikolay Tzaribachev3,
Manfred Wehrmann4,
Claus D. Claussen1 and
Jan Fritz1,5
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.
Received March 1, 2007;
accepted after revision August 7, 2007.
Address correspondence to M. Horger.
Abstract
OBJECTIVE. Our objective was to describe the spectrum of MRI
features in patients with deep and generalized morphea.
CONCLUSION. Imaging features of morphea are not specific and usually
overlap with those of other disorders involving the skin, fascia, and
musculature, such as some types of fasciitis, myositis, and so forth.
Nevertheless, the imaging features of morphea reflect pathomorphologic changes
of this rare disorder and enable a complete assessment of the disease extent,
including depth of infiltration and disease activity.
Keywords: deep morphea • generalized morphea • localized scleroderma • morphea • MRI
Introduction
Localized scleroderma, often termed "morphea" in the
dermatology literature, refers to a number of autoimmune conditions
characterized by skin thickening and increased collagen deposition. It is the
most common form of scleroderma, occurs generally in young adults, and has a
predominance in the female sex.
In morphea, the lesions are usually limited to the skin and subcutaneous
fatty tissue, but they can extend over muscular fascia, muscle tissue,
tendons, joint synovia, and even bone marrow. Other symptoms such as
arthralgia, synovitis, and infrequent ipsilateral uveitis, or even Raynaud's
phenomenon, sometimes accompany skin involvement. Visceral involvement,
affecting pulmonary function or esophageal motility, is generally considered
an attribute of systemic sclerosis and is rare in patients with morphea.
Therefore, prognosis of patients with morphea is usually good
[1,
2]. Histopathologic examination
reveals different degrees of collagenization of the dermis and extension of
fibrous tissue into the subcutaneous fat, musculature, fascia, and, rarely,
late in the course of the disease, into the bone marrow. However, depth of
infiltration by collagen bundles is expected mainly in the deep and
generalized types of morphea.
Autoimmune abnormalities of localized scleroderma have been well recognized
during the past two decades, and recently this disease has been considered to
have an autoimmune background because of the high frequency of antinuclear
antibodies encountered [3].
According to the Mayo Clinic classification, there are additional subtypes
of morphea: plaque morphea, linear morphea, bullous morphea, deep morphea
(including disabling pansclerotic morphea of children), and generalized
morphea (including eosinophilic fasciitis)
[4].
Radiologic diagnosis aims to determine the depth of collagen infiltration
involving the musculature, synovia, and bone marrow; the degree of activity of
this disorder; and related abnormalities such as polyarticular inflammation.
Imaging of morphea shows no specific findings, but involvement of the skin,
subcutaneous fatty tissue, and muscle fasciae can be easily recognized on
imaging, especially MRI, which is mandatory for accurate classification and
treatment.
Our objective was to describe the spectrum of imaging features in patients
with deep and generalized morphea.
Clinical Features in Generalized and Deep Morphea
Generalized morphea is the most severe form of localized morphea
[5,
6]. The following clinical
diagnostic criteria for this subtype of morphea must be fulfilled: four or
more lesions larger than 3 cm in diameter or involvement of two or more of the
seven body areas (the head and neck, the right and left upper extremities, the
anterior and posterior trunks, and the right and left lower extremities). In
these patients, the limbs are primarily involved, followed by the trunk and
the head. The acral areas are usually spared, contrary to systemic sclerosis
[1].
As opposed to generalized morphea, deep morphea is confined to a single or
a few anatomic areas. Dermatologic examination shows diffuse binding down of
skin over the extremities of the trunk, which is more prominent in the
extremities than in the trunk. Clinically, nodular or more keloidlike skin
changes are seen. The muscles are also firm at palpation. The induration may
extend into the deep tissue and lead to fibrosis and fixation of paraarticular
structures, which can interfere with joint motion and cause severe disability.
Reduced elasticity, vascular dysfunction, and increased vulnerability of the
skin also favor the development of ulcers. Later in the course of the disease,
progressive limb atrophy and contractures occur
[5,
7,
8].
Histologic Features in Generalized and Deep Morphea
Generalized morphea and deep morphea are characterized by sclerosis in the
septal portions of the subcutaneous fatty tissue and the deeper layers of the
dermis (Fig. 1A,
1B). After the inflammatory
phase, extensive sclerosis and hyalinization extend into the underlying
fascia. During the course of the disease, even the underlying muscles,
tendons, synovia, and bone might be involved in the process of replacement of
the differentiated tissue by collagen bundles
[7].
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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).
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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).
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Imaging Features in Generalized and Deep Morphea
Among the major imaging techniques (radiography, sonography, CT, MRI, and
scintigraphy), MRI plays a pivotal role. MRI findings in the inflammatory
stage of this disorder consist of thickening of the dermis and infiltration of
the subcutaneous fatty tissue with an increase in signal intensity on STIR
sequences in the adult (Fig.
2A) and contrast-enhanced T1-weighted images
(Fig. 2B), whereas hypointense
signal is seen on unenhanced T1-weighted images
(Fig. 2C). Beneath the
infiltrated skin, collagen tissue involves fascia and musculature, showing
different degrees of infiltration expressed similarly by increased signal
intensity on STIR (Fig. 3A) and
contrast-enhanced T1-weighted images and signal hypointensity on unenhanced
T1-weighted images (Fig.
3B).
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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.
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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.
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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.
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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).
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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).
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In time, the bone becomes involved, showing particular MRI features. A
bandlike intense signal is seen on T2-weighted (Figs.
3C and
3D) and contrast-enhanced
(Fig. 3E) T1-weighted images,
usually following the sub-cortical bone. On both T1- and T2-weighted images,
the signal is usually opposite that of normal bone marrow (Figs.
3A and
3B). Correspondingly,
hypersclerosis is seen on late follow-up radiographs. Bone biopsy is of
limited value because the disease has no characteristic pathologic features,
but biopsy is sometimes used to exclude tumor.
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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.
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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.
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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).
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After skin induration and contractures, malalignment of the fingers and
toes occurs, as shown on radiographs (Fig.
4A) or MR images. Mild joint and tendon sheath synovitis is
usually found in these patients and can be well documented on MRI (Figs.
4B,
4C, and
5). Despite generalized
synovial inflammation, bone erosions are not expected in patients with
morphea. Synovial enhancement and thickening is less aggressive than other
rheumatic and connective tissue disorders. Cutaneous, muscular, fascial, and
even osseous changes are in part reversible in patients with a favorable
response to therapy—for example, after chemotherapy with subsequent
autologous hematopoietic stem cell transplantation (HSCT) (Fig.
6A,
6B).
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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.
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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.
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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).
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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).
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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.
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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.
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As with generalized morphea (Fig.
7A,
7B,
7C,
7D,
7E), in deep morphea and its
subtypes (subcutaneous morphea, morphea profunda, disabling pansclerotic
morphea, and eosinophilic fasciitis), depth of infiltration can be better
assessed by imaging techniques (especially MRI) than by clinical inspection
because of the impediment of skin induration (Fig.
8A,
8B,
8C). This is especially true
in the diagnosis of trunk involvement by morphea. For that reason, signal
intensity depends on the degree of disease activity
[9].
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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.
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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.
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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.
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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.
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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).
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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).
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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.
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Although fascial thickening is present in most patients with morphea, its
extent is more generalized in the eosinophilic fasciitis subtype (Shulman's
syndrome), which is characterized also by peripheral eosinophilia,
hypergammaglobulinemia, and an elevated sedimentation rate accompanying skin
findings of scleroderma (Figs.
9 and
10A,
10B).
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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.
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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.
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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).
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Differential Diagnoses
Conditions that mimic morphea include mainly connective tissue diseases
with skin, fascial, and musculature involvement such as systemic sclerosis,
which should first be excluded in all patients with localized morphea, and
dermatomyositis, systemic lupus erythematosus, overlapping syndromes, and
other forms of fasciitis [10]
(Fig. 11A,
11B). Unlike morphea,
dermatomyositis is characterized clinically by gradual onset of muscle
weakness and radiologically by muscle edema, with progression of the latter to
fatty infiltration. Predominance of myositis in the vastus musculature and
sparing of the biceps femoris muscle have been noted in patients with
dermatomyositis, which is different from the distribution pattern usually
found in morphea [11] (Figs.
3A,
3B,
3C,
3D,
3E,
7A,
7B,
7C,
7D,
7E, and
8A,
8B,
8C). Fasciitis is rare and
usually mild, whereas osseous infiltration has not been reported. The most
characteristic soft-tissue abnormality in dermatomyositis is calcification,
which is absent in morphea.
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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.
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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.
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Rarer differential diagnoses are pseudo-scleroderma related to inflammatory
syndromes induced by toxins, drugs, radiation, silicon, or paraffin implants
and graft-versus-host disease (GVHD) of the skin. Fasciitis in chronic GVHD
shares many clinicopathologic features with morphea, including skin edema,
induration, fascial thickening, and myopathy, but the clinical setting makes
differentiation easy [12,
13]
(Fig. 12).
Gadodiamide-associated nephrogenic systemic fibrosis has recently been
described, and this disorder should also now be included among the
differential diagnoses of morphea
[14].
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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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Bone marrow abnormalities mimicking those in morphea are expected mainly in
patients with venous stasis, chronic recurrent multifocal osteomyelitis, shin
splint, or diverse hematologic disorders (e.g., lymphoma).
Various therapeutic techniques have been reported, but controlled trials
are rare. In patients showing no depth of infiltration, improvement can be
achieved by both psoralen ultraviolet A-range photochemotherapy and
ultraviolet A-1 (narrowband ultraviolet radiation) therapy. However, fascial,
muscular, and osseous involvement generally requires systemic therapy (e.g.,
steroids in eosinophilic fasciitis). Immunosuppressive therapies such as oral
corticosteroids or methotrexate can be helpful in the inflammatory stage.
Therefore, imaging diagnosis is beneficial for the complete assessment of the
extent of disease, including depth of infiltration and disease activity, and
might also serve as a marker for appropriate response to therapy
[9].
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- Sato S, Fujimoto M, Ihn H, et al. Clinical characteristics
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- Peterson LS, Nelson AM, Su WPD. Classification of morphea
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Abstract
Introduction
