Eosinophilic fasciitis in Adult
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Synopsis
Classically, EF presents abruptly with symmetrical erythema and edema of the affected extremities, sparing the hands, feet, and face. This progresses rapidly to induration and fibrosis, which is usually accompanied by pain and joint contractures, resulting in a decreased range of motion and paresthesias. The skin in EF is often bound down, accentuating the natural planes between muscles and vasculature, and revealing linear depressions along superficial veins, causing the "groove sign." EF is seen most commonly in White individuals, and published reviews differ on sex predominance. Age of onset has a bimodal distribution, with pediatric disease occurring in childhood to adolescence and the adult form developing between ages 30 and 50 years.
In approximately 30% of patients, a history of strenuous physical activity precedes the onset. Case reports point to the possibility that certain environmental exposures, including drugs (such as immune checkpoint inhibitors), toxins, and infections (such as COVID-19) may be potential causes. In addition, thrombocytopenia, aplastic anemia, myelodysplastic syndromes, and other myeloproliferative disorders are associated with EF. Paraneoplastic EF occurs in approximately 10% of all EF patients, most often in association with hematologic malignancies. The presence of monoclonal gammopathy has been detected in 16%-33% of patients with EF.
Trunk involvement and the presence of peau d'orange have been associated with a poorer prognosis. Features that distinguish EF from deep morphea and scleroderma are a peripheral eosinophilia (in 60%-80% of patients), hypergammaglobulinemia (in 20%-70% of patients), and an absence of Raynaud phenomenon that is common to systemic sclerosis. In a retrospective study, 21 of 60 patients (35%) had concurrent plaque morphea. Antinuclear antibody (ANA) titers are normal in EF and the ESR is usually elevated.
Codes
M35.4 – Diffuse (eosinophilic) fasciitis
SNOMEDCT:
24129002 – Eosinophilic fasciitis
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Last Updated:03/15/2026
