Pain and hardening of the skin constricts shoulder movement
The patient’s condition was marked by tethering of the skin to underlying tissue.
A 32-year-old man of Indian origin presented to the family medicine clinic with pain and swelling in both upper extremities of one-month duration. He described the pain as dull and aching in nature and confined to both upper arms and shoulders. Overhead movement at both shoulders was restricted beyond 90°.
The patient was currently taking OTC acetaminophen and ibuprofen for pain with minimal relief. He was alert but in distress from the pain. The physical examination of head, ear, eye, nose, and throat was normal, as was that of the lungs, heart, and abdomen. Musculoskeletal examination revealed swelling of the upper arms and hardening and tethering of skin to underlying tissue. Movements at both shoulders, particularly abduction and lateral rotation, were limited.
Laboratory studies revealed slightly increased C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and marked eosinophilia. Stool studies were negative for parasitic infections. The patient refused a recommendation for full-thickness skin-to-muscle biopsy.
The patient was treated empirically for possible eosinophilic fasciitis (EF) with prednisone for two months. Re-evaluation within two weeks demonstrated significant symptomatic improvement along with a decline in eosinophilia. Subsequent follow-up evaluation revealed complete resolution of symptoms and eosinophilia.
EF was first described by Shulman in 1974 as an inflammatory disease characterized by diffuse fasciitis and eosinophilia.1 It is an uncommon connective-tissue disorder that manifests primarily as sclerodermalike syndrome associated with symmetrical swelling, induration, and thickening of the skin and subcutaneous tissue of the proximal and distal extremities.
The incidence of this rare condition is not known, and there is no clear consensus about the demographics. The mean age of onset is between 30 and 50 years.2 However, the disease has been reported in children and elderly patients as well.3
EF has varying clinical presentations. Most patients typically present with swelling and induration of the arms and legs with skin thickening.4 The disease usually progresses from edema of the extremities to woody induration with skin tightness. Patients may also have joint pain with associated synovitis. Some patients have presented with progressive weakness, pain, and stiffness of both upper and lower extremities.2
The most common extracutaneous manifestation is the involvement of joints resulting in inflammatory arthritis, contracture, and carpal tunnel syndrome.5 Reactive lung disease, pleural effusions, and multiple myeloma have also been reported as extracutaneous manifestations of EF.6,7
Diagnosis of EF is often delayed due to overlapping features with other connective-tissue diseases (e.g., scleroderma, polymyositis, and hypereosinophilic syndrome). Confirmatory diagnosis is achieved by full-thickness skin-to-muscle biopsy,4 which shows inflammation and thickening of collagen bundles with infiltration of lymphocytes and plasma cells in superficial muscle fascia. Eosinophils are occasionally observed in biopsy but are not necessary to make the diagnosis. When used as a diagnostic tool, MRI shows fascia thickening and helps identify the suitable biopsy site, which facilitates accurate and timely diagnosis of EF.8