Eosinophilic fasciitis
Updates to Article Attributes
Eosinophilic fasciitis (EF),also known as Shulman syndrome, is an uncommon connective tissue disorder.
Epidemiology
It can potentially present at age. There is a recognized female predilection 3-4.
Clinical presentation
Patients typically present with pronounced extremity oedema and skin skin induration.
Clinical features include
- peripheral eosinophilia
- hypergammaglobulinemia
- elevated erythrocyte
sedimentationsedimentation rate (ESR)/C reactive protein (CRP) - scleroderma like cutaneous changes
Location
Eodinophilic fasciitis tends to involve the limbs.
Radiographic features
MRI
Tends to demonstrate muscular fascial plane thickening without any myositis. Changes are generally symmetrical. Signal changes include 1,3:
- T2 FS/STIR: high signal compared to muscle involving fascial planes
-
T1 C+ (Gd): shows
fascialfascicle enhancement
Treatment and prognosis
Tends to respond to corticosteroids with excellent response.
History and etymology
First described by L E Shulman et al. in 1975 2.
Differential diagnosis
Imaging differential considerations include:
-
necrotising fasciitis: tends to have accompanying
fluidfluid/gas collections/abscess formation, cellulitis and muscle inflammation
-<p><strong>Eosinophilic fasciitis (EF)</strong>,<strong> </strong>also known as <strong>Shulman syndrome</strong>, is an uncommon connective tissue disorder.</p><h4>Epidemiology</h4><p>It can potentially present at age. There is a recognized female predilection <sup>3-4</sup>.</p><h4>Clinical presentation</h4><p>Patients typically present with pronounced extremity oedema and skin induration. </p><p>Clinical features include</p><ul>- +<p><strong>Eosinophilic fasciitis (EF)</strong>,<strong> </strong>also known as <strong>Shulman syndrome</strong>, is an uncommon connective tissue disorder.</p><h4>Epidemiology</h4><p>It can potentially present at age. There is a recognized female predilection <sup>3-4</sup>.</p><h4>Clinical presentation</h4><p>Patients typically present with pronounced extremity oedema and skin induration. </p><p>Clinical features include</p><ul>
-<li>elevated erythrocyte sedimentation rate (ESR)/C reactive protein (CRP)</li>- +<li>elevated erythrocyte sedimentation rate (ESR)/C reactive protein (CRP)</li>
-<strong>T1 C+ (Gd)</strong>: shows fascial enhancement</li>-</ul><h4>Treatment and prognosis</h4><p>Tends to respond to corticosteroids with excellent response.</p><h4>History and etymology</h4><p>First described by <strong>L E Shulman</strong> et al in 1975 <sup>2</sup>.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include:</p><ul><li>-<a href="/articles/necrotising-fasciitis">necrotising fasciitis</a>: tends to have accompanying fluid/gas collections/abscess formation, cellulitis and muscle inflammation</li></ul>- +<strong>T1 C+ (Gd)</strong>: shows fascicle enhancement</li>
- +</ul><h4>Treatment and prognosis</h4><p>Tends to respond to corticosteroids with excellent response.</p><h4>History and etymology</h4><p>First described by <strong>L E Shulman</strong> et al. in 1975 <sup>2</sup>.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include:</p><ul><li>
- +<a href="/articles/necrotising-fasciitis">necrotising fasciitis</a>: tends to have accompanying fluid/gas collections/abscess formation, cellulitis and muscle inflammation</li></ul>