Eosinophilic fasciitis

Eosinophilic fasciitis (EF), also known as Shulman syndrome or diffuse fasciitis with eosinophilia, is a rare connective tissue disorder characterized by symmetric inflammation and thickening of the fascia (the connective tissue surrounding muscles, blood vessels, and nerves beneath the skin). The condition primarily affects the extremities, particularly the arms and legs, and typically presents with sudden onset of painful swelling, skin induration (hardening), and a characteristic 'peau d'orange' (orange peel) appearance of the skin. The affected limbs may develop a woody texture, and joint contractures can occur as the disease progresses, limiting range of motion. Peripheral blood eosinophilia and elevated erythrocyte sedimentation rate (ESR) are common laboratory findings. The onset is frequently preceded by strenuous physical activity. Eosinophilic fasciitis affects the musculoskeletal and dermatologic systems primarily. Unlike systemic sclerosis (scleroderma), which it can resemble, EF typically spares the hands, feet, and face, and Raynaud's phenomenon is generally absent. Internal organ involvement is uncommon, though some patients may develop hematologic abnormalities, including aplastic anemia, thrombocytopenia, or, rarely, hematologic malignancies. A full-thickness biopsy extending from skin to muscle is the gold standard for diagnosis, revealing inflammation and thickening of the fascia with eosinophilic infiltration. The mainstay of treatment is systemic corticosteroids, which are effective in the majority of patients, particularly when initiated early in the disease course. Many patients respond well to moderate-to-high dose corticosteroids (such as prednisone), with gradual tapering over months. For patients who do not respond adequately to corticosteroids or who relapse, immunosuppressive agents such as methotrexate, mycophenolate mofetil, or hydroxychloroquine may be used as steroid-sparing therapies. Physical therapy is an important adjunct to help maintain range of motion and prevent contractures. The prognosis is generally favorable, with many patients achieving partial or complete remission, though some may experience a chronic or relapsing course.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Eosinophilic fasciitis