Eosinophilic fasciitis (Shulman syndrome) is a rare scleroderma-like disorder with inflammation and fibrosis of the deep fascia, sparing the hands and face.

Mean onset 40 to 50 years; men and women equally affected.
Triggers in a third: strenuous exercise or trauma, L-tryptophan, statins, radiotherapy, infections (Borrelia).
Associations: morphea, haematological disease (aplastic anaemia, myelodysplasia, lymphoma), thyroiditis.
Course: partial or complete remission in most with treatment; relapse and contractures possible.

Peripheral eosinophilia (in 60 to 90%, may be transient), raised ESR, hypergammaglobulinaemia, mild CK normal.
MRI: fascial thickening and enhancement with T2 hyperintensity.
Full thickness skin-to-muscle biopsy (deep): thickened fascia with inflammatory infiltrate (lymphocytes, plasma cells, eosinophils) and fibrosis.
Antinuclear antibodies usually negative; exclude systemic sclerosis (capillaroscopy, scleroderma antibodies), nephrogenic systemic fibrosis, scleredema, hypereosinophilic syndrome, drug-induced.
Peripheral blood film, marrow when cytopenias or monoclonal protein; screen for haematological malignancy.

Prednisolone 0.5 to 1 mg/kg/day (maximum 60 mg) for several months with slow taper over 6 to 12 months; methylprednisolone 500 to 1000 mg IV for 3 days in severe disease. Response in 70% within weeks.
Steroid-sparing agent early: methotrexate 15 to 25 mg weekly (improves response and reduces relapse), mycophenolate mofetil 1 to 1.5 g twice daily, azathioprine, ciclosporin 3 to 5 mg/kg.
Refractory: IVIG 2 g/kg, tocilizumab 162 mg weekly, rituximab, infliximab, UVA1 phototherapy, hydroxychloroquine.
Physiotherapy early to prevent contractures; surgical release of contractures if fixed.
Pneumocystis prophylaxis and bone protection with prolonged steroids.
Monitor for haematological disease with annual counts.

Eosinophilic Fasciitis

Overview

Clinical Features

Diagnosis

Management