ConditionBones, joints & muscles
Eosinophilic Fasciitis
Eosinophilic fasciitis is a rare inflammatory condition in which the fascia, the connective tissue sheet beneath the skin and fat, becomes inflamed and thickened. It typically starts with painful swelling of both arms or legs, often after unusually strenuous exercise, followed over weeks by hardening of the skin, a "dimpled orange peel" look and stiff joints. Blood tests often show high eosinophils. Unlike scleroderma, it usually spares the hands, feet and face and rarely affects internal organs. Steroids and methotrexate are the main treatments.
Also called: Shulman syndrome, Shulman's syndrome, diffuse fasciitis with eosinophilia
At a glance
- What it is
- Inflammation and thickening of the fascia beneath the skin, usually of the limbs.
- Who gets it
- Adults, most often aged 30 to 60; men and women similarly.
- Typical onset
- Sudden painful swelling of both arms and legs, sometimes after strenuous activity.
- Key signs
- Woody hardening, orange-peel skin, the "groove sign" along veins, joint stiffness; hands, feet and face usually spared.
- Diagnosis
- MRI and a deep biopsy from skin to muscle; blood eosinophilia.
- Treatment
- Corticosteroids, often with methotrexate; physical therapy to prevent contractures.
When to get help
See a doctor within a day or two
- Rapidly spreading swelling and hardening of the arms or legs with pain
- New tiredness, bruising, bleeding or frequent infections in someone with eosinophilic fasciitis (rare blood disorders can occur)
Book a routine appointment
- Swelling followed by skin tightening of the forearms or calves over weeks
- Joints that are becoming stiff or cannot fully straighten
- Numbness or tingling in the thumb and fingers with arm involvement
- Thick, tight skin of the limbs with no Raynaud's phenomenon or finger changes
What happens in eosinophilic fasciitis
The fascia wraps around muscles. In eosinophilic fasciitis, immune cells, including eosinophils (a type of white blood cell), infiltrate the fascia, causing inflammation that is later replaced by scarring (fibrosis). The thickened fascia tethers the skin and fat to deeper tissues, producing the characteristic hardening and puckering and limiting movement.
Symptoms
Early phase
- sudden, symmetrical swelling and tenderness of the forearms, upper arms, thighs or calves
- pain and a feeling of tightness
- fatigue, sometimes low-grade fever and weight loss
Later phase
- woody, hard skin that cannot be pinched
- peau d'orange: an orange-peel, dimpled appearance
- groove sign: linear depressions along the course of superficial veins, best seen when the limb is raised
- reduced movement and contractures of the elbows, wrists, knees and ankles
- carpal tunnel syndrome from thickening at the wrist
- patches resembling morphea (localized scleroderma) in some people
The hands, feet and face are usually spared.
Causes and triggers
The cause is unknown. Reported triggers include:
- intense or unaccustomed physical exertion (in about a third to half of cases)
- trauma
- certain medicines, including some cancer immunotherapies
- infections such as Borrelia in some reports
It is associated with blood disorders in a minority, including aplastic anemia, thrombocytopenia, lymphomas and other bone marrow conditions, so blood counts are monitored.
Eosinophilic fasciitis vs systemic sclerosis
| Eosinophilic fasciitis | Systemic sclerosis | |
|---|---|---|
| Onset | Sudden, over days to weeks | Gradual |
| Hands and fingers | Usually spared | Finger thickening (sclerodactyly), ulcers |
| Raynaud's phenomenon | Usually absent | Present in almost all |
| Face | Spared | Often tight skin around the mouth |
| Internal organs | Rarely involved | Lungs, gut, kidneys, heart often involved |
| Blood eosinophils | Often high, early | Usually normal |
| Nailfold capillaries | Normal | Abnormal |
Diagnosis
- Examination: symmetrical limb induration, groove sign, sparing of hands and face.
- Blood tests: a raised eosinophil count (eosinophilia) in most people early on, raised ESR, and high immunoglobulin levels. Eosinophils may fall quickly and are not required for diagnosis.
- MRI: shows thickening and inflammation of the fascia and helps choose a biopsy site and monitor response.
- Full-thickness biopsy: a deep sample from skin through fat and fascia to muscle confirms inflammation and thickening of the fascia.
- Tests to exclude other causes: antibodies for scleroderma, nailfold capillaroscopy, blood count and sometimes bone marrow examination.
Treatment
- Corticosteroids: high-dose prednisone (sometimes started with intravenous methylprednisolone) is first-line, then gradually tapered over months.
- Methotrexate: commonly added to improve response and reduce steroid use.
- Other options for resistant disease: mycophenolate, hydroxychloroquine, sirolimus, rituximab, IVIG, JAK inhibitors or other biologics, mostly based on case series.
- Physical and occupational therapy: essential to maintain range of motion and prevent contractures.
- Surgery: carpal tunnel release or release of fixed contractures in some.
Early treatment gives the best results. Avoiding strenuous exertion during active disease is often advised.
Outlook
Many people improve substantially over months to a few years with treatment, and some recover fully. Others are left with skin thickening or joint contractures. Factors linked to a less favorable outcome include morphea-like skin lesions, involvement of the trunk, younger age at onset and delayed treatment. Monitoring for associated blood disorders continues.
Frequently asked questions
What is eosinophilic fasciitis?
Eosinophilic fasciitis is a rare condition in which the fascia under the skin becomes inflamed and thickened, causing painful swelling and then hardening of the arms and legs. It is also called Shulman syndrome. It often follows strenuous exercise, but the cause is unknown.
What is the groove sign?
The groove sign is a visible linear depression along the path of superficial veins in thickened skin, seen especially when the arm or leg is raised. It occurs because the veins are tethered while the surrounding tissue is thickened, and is characteristic of eosinophilic fasciitis.
How is eosinophilic fasciitis different from scleroderma?
Eosinophilic fasciitis usually starts suddenly, spares the hands, fingers and face, lacks Raynaud's phenomenon and rarely affects internal organs. Systemic sclerosis typically involves the fingers, causes Raynaud's and often affects the lungs, gut and kidneys.
How is eosinophilic fasciitis treated?
The main treatment is corticosteroids, often combined with methotrexate. Physical therapy is important to keep joints moving. Other immune-suppressing medicines are used if the response is inadequate.
Can eosinophilic fasciitis go away?
Yes, in many cases. Some people go into remission, especially with early treatment, and a few improve without treatment. Others have lasting skin tightness or joint stiffness. Recovery often takes months to years.
Sources
This guide was written from current patient and clinical guidance, including:
- NIH Genetic and Rare Diseases Information Center (GARD): Eosinophilic fasciitis
- National Organization for Rare Disorders (NORD): Eosinophilic fasciitis
- American College of Rheumatology: Scleroderma and related disorders
- DermNet: Eosinophilic fasciitis
- Journal of the American Academy of Dermatology: Eosinophilic fasciitis review