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Eosinophilic fasciitis in Child
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Eosinophilic fasciitis in Child

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Contributors: Edward Li PhD, Zachary Skabelund MD, Susan Burgin MD
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Synopsis

Eosinophilic fasciitis : Eosinophilia, Erythema, Groove sign, Joint contractures, Peau d'orange, Symmetric extremities distribution, Arm edema, Leg edema, Induration
Eosinophilic fasciitis (EF), also known as Schulman syndrome or diffuse fasciitis with eosinophilia, is a rare disorder characterized by abrupt and symmetric edema, inflammation, and fibrosis of the muscle fascia. The etiology is unknown, although approximately half of EF patients experience trauma or intense exercise preceding the onset of disease. While EF is especially rare in children, it can and has been noted to occur in children as young as 1 year old. A younger age of onset is associated with a greater risk of progression to cutaneous fibrosis and joint contractures. 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 have 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. The face is generally spared. The hands and feet are affected more frequently in children than adults.

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 Reviewed:03/11/2019
Last Updated:01/16/2022
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Eosinophilic fasciitis in Child
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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.

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