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Eosinophilic fasciitis in Child
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Eosinophilic fasciitis in Child

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Contributors: Zachary Skabelund MD, Susan Burgin MD
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Synopsis

Eosinophilic fasciitis (EF, also known as Schulman's syndrome or diffuse fasciitis with eosinophilia) is a rare disorder characterized by abrupt and symmetric fibrosis of the muscle fascia. The etiology is unknown, though about half of patients experience trauma or intense exercise preceding the onset of disease. Although it is especially rare in children, EF can be seen throughout the entire pediatric age range and has been noted to occur in children as young as 1 year. In contrast to findings among adults with EF, there is a female predominance among children, with a female-to-male ratio of 3:1. Individuals of Northern European descent are most often reported to suffer from this condition.

EF presents with symmetric tenderness, erythema, and edema of affected extremities, with induration of the underlying muscle fascia and subcutaneous tissue occurring over days to weeks. The skin becomes tethered, giving rise to a characteristic dimpling and peau d'orange appearance. Elevation of the affected limb will cause a depression along superficial veins, leading to distinctive furrowing referred to as the groove sign. Constitutional symptoms, such as fatigue and weight loss, and local hypopigmentation, hair loss, and scaling may be seen. Secondary arthritis, joint contractures, and nerve compression may occur. Arthritis is less commonly seen among children than adults.

The disease is typically symmetric and preferentially affects the extremities, especially the forearms and calves, though the hands, feet, trunk, and neck may also be involved. The face is generally spared. The hands and feet have been found to be affected in a greater percentage of children than adults. Young children are at higher risk of developing residual scleroderma-like cutaneous fibrosis.

A unique variant has been described in children, in which patients present with painless contractures and a biopsy diagnostic of EF without the typical progression of swelling, pain, and characteristic dermatologic findings.

Plaque morphea has been seen to present concurrently with EF.

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

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: 06/07/2016
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Eosinophilic fasciitis in Child
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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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