Specialty rheumatology ICD-9-CM 728.89 DiseasesDB 29427 | ICD-10 M35.4 OMIM 226350 MedlinePlus 000447 | |
Eosinophilic fasciitis (/ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtᵻs, ˌiːoʊ-, -ˌfæsi-/), also known as "Shulman's syndrome", is a form of fasciitis, the inflammatory diseases that affect the fascia, the connective tissues surrounding muscles, blood vessels and nerves. Unlike other diseases in that category, it is limited to the arms and legs, and usually resolves itself, although some cases require corticosteroids, and some cases are associated with aplastic anemia.
Contents
The presentation of eosinophilic fasciitis is similar to scleroderma or systemic sclerosis. However, unlike scleroderma, it affects the fascia, not the skin (dermis). The characteristic and severe effects of scleroderma and systemic sclerosis, such as Raynaud's syndrome, involvement of the extremities, prominent small blood vessels (telangiectasia), and visceral changes such as swallowing problems, are absent.
It was first characterized in 1974, and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.
Several cases have been reported after strenuous exercise.
Symptoms
As it is a rare disease, a clear set of symptoms is difficult to define. Usually, patients show severe pain and swelling is reported but clinical presentations vary. It can have an 'orange peel' like appearance. Less common features are joint pain and carpal tunnel syndrome.
Treatment
Common treatments include corticosteroids such as prednisone, though other medications such as hydroxychloroquine have also been used. The prognosis is usually good in the case of an early treatment if there is no visceral involvement.
Diagnosis
The key to diagnosis is skin changes combined with blood eosinophilia but the most accurate test is a skin, fascia and muscle biopsy.
Epidemiology
Typical age of onset is around 40 to 50 years. It is not clear whether it is more common in women than men - patient numbers are small and some studies report a preponderance of men and others women. It is also found in children.