Eosinophilic fasciitis
Evidence: Unconfirmed
Names
Eosinophilic fasciitis
Pansclerotic morphea with deep fascial involvement
Shulman syndrome
Description
Eosinophilic fasciitis is a rare, scleroderma-like, autoimmune rheumatic disorder in which the skin and tissue (the fascia, fibrous tissue that separates different layers of tissues under the skin) that lies beneath the skin become painfully inflamed and swollen and gradually hardens.
It affects the forearms, the upper arms, the lower legs, the thighs, and the trunk (in order of decreasing frequency). The disease is considered by some to be a deep variant of the skin condition, morphea, and is also known as "pansclerotic morphea with deep fascial involvement.
Patients usually present suddenly with painful, tender, swollen and red extremities. Within weeks to months patients develop stiffness and affected skin becomes indurated, creating a characteristic orange-peel appearance over the surfaces of the extremities. In severely affected areas, the skin and the subcutaneous tissue are bound tightly to the underlying muscle. This creates a woody-type appearance.
The presentation of eosinophilic fasciitis is similar to scleroderma or systemic sclerosis. However, unlike scleroderma, it affects the fascia, not the skin (dermis). Unlike scleroderma and systemic sclerosis, effects such as Raynaud's phenomenon, 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.
Patient Groups
None found.
Bloggers
None found.
Prevalence
No data found. Most reports state that the disease is very rare, with hundreds of cases. However, there are already 4 participants in the Autoimmune Registry with the disease, which suggests it is potentially more common than published reports indicate.
Typical Age of onset
Most cases start between the ages of 30 and 60 years, with men and women equally affected (note: we found no studies to support this).
Symptoms
Sudden onset with pain, tenderness, redness, and swelling in the extremities
Over weeks and months patients, skin feels stiff and hard (indurated)
An “orange-peel” appearance on the skin of the extremities
In severely affected areas, the skin has a woody-type appearance
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.
