Eosinophilic fasciitis, also known as Shulman disease/syndrome, is an uncommon connective tissue disorder.
It can potentially present at any age. There is a recognized female predilection .
Patients typically present with pronounced extremity edema and skin induration.
Clinical features include
- peripheral eosinophilia
- elevated erythrocyte sedimentation rate (ESR) / C-reactive protein (CRP)
- scleroderma-like cutaneous changes
Eosinophilic fasciitis tends to involve the limbs.
MRI demonstrates muscular fascial plane thickening without any myositis. Changes are generally symmetrical. Signal changes include the following :
- T2 FS/STIR: high signal compared to muscle involving fascial planes
- T1 C+ (Gd): shows fascicle enhancement
Treatment and prognosis
Eosinophilic fasciitis tends to respond to corticosteroids with excellent response.
History and etymology
The entity was first described by L E Shulman et al. in 1975 .
Imaging differential considerations include
- necrotizing fasciitis: tends to have accompanying fluid/gas collections/abscess formation, cellulitis and muscle inflammation