eosinophilic fasciitis

Eosinophilic fasciitis, also known as Shulman disease/syndrome, is an uncommon connective tissue disorder.

Epidemiology

It can potentially present at any age. There is a recognized female predilection .

Clinical presentation

Patients typically present with pronounced extremity edema and skin induration.

Clinical features include

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

Location

Eosinophilic fasciitis tends to involve the limbs.

Radiographic features

MRI

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 .

Differential diagnosis

Imaging differential considerations include

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

Siehe auch:

• Systemische Sklerodermie
• Nekrotisierende Fasziitis

und weiter:

• Fasziitis