Eosinophilic fasciitis
Inflammatory disease of fascia with eosinophilia and limb involvement.
Eosinophilic fasciitis, also known as Shulman's syndrome or diffuse fasciitis with eosinophilia, is an inflammatory disease affecting the fascia, connective tissues, muscles, blood vessels, and nerves. It is typically self-limited and confined to the arms and legs, though it can require corticosteroid treatment and some cases are associated with aplastic anemia. The condition was first characterized in 1974, but it remains uncertain whether it is a distinct condition or a variant of another syndrome.
Quick Facts
- Male to female ratio
- 1.5:1
- Typical age of onset
- 20 to 60 years
- Number of reported cases
- about 100
Facts from the source article.
Did You Know?
- First-line treatment is oral corticosteroid therapy, tapered over 1–2 years.
- Second-line therapies include DMARDs such as hydroxychloroquine, methotrexate, cyclosporine, and cyclophosphamide.
- Prognosis is usually good with early treatment if there is no visceral involvement.
Signs and symptoms
Pain and swelling in the arms and legs are the main signs, often starting with symmetrical thickening and redness on the forearms and lower legs while leaving the hands and feet untouched. As the condition advances, the skin can take on a dimpled, orange-peel texture. Some people also experience fever, tiredness, skin hardening, joint aches, and carpal tunnel syndrome from nerve compression. Because this disorder is uncommon and symptoms differ widely, no single set of features defines it.
Diagnosis
The first step is to exclude other conditions such as systemic sclerosis. Key diagnostic features include skin changes combined with eosinophilia, but the most accurate test is a biopsy of skin, fascia, and muscle. Imaging like MRI shows thickening of the fascia. Definitive diagnosis requires meeting the major criterion and at least one of two minor criteria.
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