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Eosinophilic fasciitis in Adult
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Eosinophilic fasciitis in Adult

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Contributors: Edward Li PhD, 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 the abrupt fibrosis of the fascia overlying the musculature of the body. The disease is very rare, with only about 250 cases reported in the medical literature.

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

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.

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

  • Scleroderma – A more superficial fibrotic connective tissue disease that is associated with Raynaud phenomenon and internal visceral involvement.
  • 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 Reviewed: 03/12/2019
Last Updated: 04/22/2019
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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
Clinical image of Eosinophilic fasciitis
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