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

Eodinophilic fasciitis tends to involve the limbs.

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 fascicle 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
Tags: cases, cases
Synonyms or Alternate Spellings:
  • Shulman syndrome
  • Shulman's syndrome

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