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Eosinophilic fasciitis in Adult
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Eosinophilic fasciitis in Adult

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Contributors: Susan Burgin MD, Misha Miller MD, Whitney A. High MD, JD, MEng
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Synopsis

Eosinophilic fasciitis (EF), also known as Schulman syndrome, is a fibrosing disorder characterized by abrupt fibrosis of the fascia overlying the musculature of the body. The cause of the disease is unknown. The condition results in woody induration of the soft tissue of the body, particularly the extremities, and it is accompanied by pain and joint contractures resulting in a decreased range of motion and paresthesias.

The disease is very rare, with only about 250 cases reported in the medical literature. Individuals of Northern European descent have been most often reported to suffer from EF. The disease has occurred in patients of all ages, from childhood to the elderly, but most patients are middle aged.

Classically, EF presents with symmetrical edema and aching of the affected extremities, which progress rapidly to fibrosis, induration, and a peau d'orange appearance. The skin in EF is often bound down, and this accentuates the natural planes between muscles, giving a muscular or grooved appearance. Subacute and more insidious forms of EF also exist. In about one-quarter of patients, trauma or intense exercise precedes the onset of the disease.

While some authorities believe that EF overlaps with deep morphea and scleroderma, features that distinguish EF are a peripheral eosinophilia (in 60% to 80% of patients), hypergammaglobulinemia (in 20% to 70% of patients), and an absence of Raynaud phenomenon, common to scleroderma. In a retrospective study, 21 of 60 patients (35%) had concurrent plaque morphea.

Immune-mediated anemia, thrombocytopenia, pure red cell aplasia, aplastic anemia, myelodysplastic syndromes, and lymphoproliferative processes (T- or B-cell lymphoma, multiple myeloma) have occurred concomitantly in few reported cases of EF.

For more information, see OMIM.

Codes

ICD10CM:
M35.4 – Diffuse (eosinophilic) fasciitis

SNOMEDCT:
24129002 – Fasciitis with eosinophilia syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The differential diagnosis of EF includes the following:
  • Scleroderma – a more superficial fibrotic connective tissue disease that is associated with Raynaud phenomenon and internal visceral involvement, such as esophageal dysfunction
  • Morphea – a form of cutaneously limited scleroderma characterized by isolated plaques of the skin; it is usually not as symmetrical, extensive or abrupt as EF
  • Scleromyxedema – presents with papules coalescing into indurated plaques on the extremities and is associated with paraproteinemia as well, but biopsy shows a hypercellular and myxedematous superficial fibrosis rather than a deep fibrosis of the fascia
  • Nephrogenic systemic fibrosis – occurs in the setting of significant kidney disease and is highly associated with the administration of gadolinium-based contrast agents during periods of renal insufficiency
  • Eosinophilia-myalgia syndrome / toxic oil syndrome – in the 1980s and early 1990s, contaminated rapeseed oil and contaminated tryptophan supplements resulted in a disease presentation similar to EF that caused erythema, edema, fibrosis, and joint contractures, but the illnesses disappeared with identification of the contaminated ingestants

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 03/29/2017
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Eosinophilic fasciitis in Adult
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Eosinophilic fasciitis : Joint contractures, Lower extremity edema, Peau d'orange, Limb pain, Arm edema, Hardened skin
Clinical image of Eosinophilic fasciitis
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