Fact-sheet: Eosinophilic fasciitis - schulman disease
Updated on 04/05/2016 at 12:13 PM
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Definition
-Eosinophilic fasciitis, first described in 1974 by Dr Shulman.
-Rare entity, may occur at any age, more common in adulthood (20-50 years)
-Poorly understood etiology. Unusual intense physical exertion found in 30 to 46% of cases.
Clinical features
Cutaneous manifestations:
-The first to appear.
-Symmetric edema, sometimes painful and inflammatory, of the extremities, with centripetal progression.
-Sclerodermiform subcutaneous induration (thickened, board-like skin, not mobile and impossible to pinch)
-Predominant involvement of the extremities but may extend to the neck and trunk. Fingers, feet and face frequently spared.
-50% of cases: orange-peel appearance of the upper limbs with venous grooving instead of prominence: "groove" sign
Musculoskeletal manifestations:
-Common at the initial stage.
-Mono-, oligo- or polyarticular arthritis of the hands, wrists and Knees
-Early myalgias, sometimes severe
Laboratory findings
Nearly constant hypereosinophilia
Radiography
Normal
Ultrasound
Fewer studies available
CT
With contrast injection: thickening of the muscle fasciae
MRI
Reference examination.
Bilateral and symmetric involvement.
Fasciae and intermuscular septa thickened, showing non-fluid T2 hyperintensity and enhancement after contrast injection.
Detection of these abnormalities is optimized by fat-suppressed sequences
Nuclear medicine
PET-CT: hypermetabolism of the affected regions
Management
Corticosteroid therapy and physical therapy
Immunosuppressive treatment in case of failure
Differential diagnosis
-Systemic sclerosis -
Eosinophilia-myalgia syndrome -
Toxic oil syndrome -
Churg-Strauss disease