Eosinophilic fasciitis

Eosinophilic fasciitis (EF), also known as Shulman syndrome, is an uncommon connective tissue disorder.

It can potentially present at age. There is a recognized female predilection 3-4.

Patients typically present with pronounced extremity oedema and skin induration. 

Clinical features include

  • peripheral eosinophilia
  • hypergammaglobulinemia
  • elevated erythrocyte sedimentation rate (ESR)/C reactive protein (CRP)
  • scleroderma like cutaneous changes
Location

Eodinophilic fasciitis tends to involve the limbs.

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 fascial enhancement

Tends to respond to corticosteroids with excellent response.

First described by L E Shulman et al in 1975 2.

Imaging differential considerations include:

  • necrotising fasciitis: tends to have accompanying fluid/gas collections/abscess formation, cellulitis and muscle inflammation
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Article Information

rID: 10777
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Shulman syndrome
  • Shulman's syndrome

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