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Eosinophilic fasciitis in Adult
Other Resources UpToDate PubMed

Eosinophilic fasciitis in Adult

Contributors: Vivian Wong MD, PhD, Edward Li PhD, Erin X. Wei MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Eosinophilic fasciitis (EF), also known as Schulman syndrome, is a rare fibrosing disorder characterized by the abrupt fibrosis of the fascia overlying the musculature of the body. 

Classically, EF presents with symmetrical edema and woody induration of the affected extremities sparing the hands, feet, and face, which progresses rapidly to fibrosis, induration, and a peau d'orange appearance. EF 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.

The cause of the disease is currently unknown. EF affects both sexes, with women more commonly affected than men. White individuals have been most commonly reported to suffer from EF. The disease has occurred in patients of all ages, from childhood to older adults, but most patients are between ages 30 and 50. A history of strenuous physical activity preceding the clinical findings of EF occurs in approximately 30% of patients. Some researchers have hypothesized that certain environmental exposures, including drugs, toxins, and infections (Borrelia burgdorferi), may be potential causes. In addition, thrombocytopenia, aplastic anemia, myelodysplastic syndromes, and other myeloproliferative disorders are associated with EF. 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 reported to be associated with a poorer prognosis. While some authorities believe that EF overlaps with deep morphea and scleroderma, features that distinguish EF 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. Erythrocyte sedimentation rate (ESR) is usually elevated.

Codes

ICD10CM:
M35.4 – Diffuse (eosinophilic) fasciitis

SNOMEDCT:
24129002 – Fasciitis with eosinophilia syndrome

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Last Reviewed:05/24/2022
Last Updated:01/08/2023
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Eosinophilic fasciitis in Adult
A medical illustration showing key findings of Eosinophilic fasciitis : Eosinophilia, Erythema, Groove sign, Joint contractures, Peau d'orange, Symmetric extremities distribution, Arm edema, Leg edema, Induration
Clinical image of Eosinophilic fasciitis - imageId=253795. Click to open in gallery.  caption: 'Skin-colored nodules with a surrounding bound-down appearance of the thigh.'
Skin-colored nodules with a surrounding bound-down appearance of the thigh.
Copyright © 2024 VisualDx®. All rights reserved.