Title:A Literature Review of Eosinophilic Fasciitis with an Illustrative Case
Volume: 13
Issue: 2
Author(s): Joyutpal Das*, Hector Chinoy, Jeremy Dick, Rachael Matthews and Mark Roberts
Affiliation:
- Neurology Department, Salford Royal NHS Foundation Trust, Stott Lane, Salford, M6 8HD,United Kingdom
Keywords:
Eosinophilic fasciitis, eosinophilia, scleroderma, ubiquitously, scleroderma, haematological.
Abstract: Eosinophilic fasciitis (EF), a rare connective tissue disorder, was first reported by Lawrence
Shulman in 1974. Since then over 300 cases have been reported worldwide. EF has variable
clinical presentations and currently does not have internationally accepted diagnostic criteria. Dermatological
features are the most ubiquitously present symptoms. It often presents with sclerodermalike
skin changes. Extracutaneous presentations, such as arthritis and carpal tunnel syndrome can
precede cutaneous changes. The most useful clinical features are the lack of Raynaud’s phenomenon,
telangiectasia and visceral involvement, differentiating it from Scleroderma. Haematological disorders,
solid tumours and autoimmune disorders are frequently associated with EF. Historically, the
presence of peripheral eosinophilia, elevated ESR and hypergammaglobulinemia were considered to
be diagnostic of EF. It is now well recognised that neither the presence of eosinophilia in the blood
nor eosinophilic infiltartion in the fascia is necessary for its diagnosis. An en bloc surgical biopsy
including skin, subcutis, fascia and muscle is the gold-standard test for diagnosing EF. Magnetic
resonance imaging helps to locate a suitable biopsy site and to monitor treatment response. Although
its underlying aetiology is unknown, there is a growing body of evidence supporting an immunological
origin. Immunosuppressive drugs are used to treat EF and the corticosteroid is the first line treatment.
A significant proportion of patients can develop permanent disabilities such as joint contractures,
tendon retraction and subdermal sclerosis. Occasionally it can be treatment refractory or have a
relapse-remitting course. We report another case of EF with a literature review.
