SHM Converge Scientific Abstract ePoster Gallery-Eosinophilic Fasciitis: The Scleroderma Mimicker

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Eosinophilic fasciitis (EF) is a rare connective tissue disease, often called a “scleroderma mimicker,” because it can cause skin tightening and hardening similar to scleroderma. It is uncommon, with only a few hundred cases reported, and pediatric cases are especially rare. EF typically affects adults, but children may have more severe disease.<br /><br />The case described a 9-year-old girl who developed two months of skin tightness, hyperpigmentation, stiffness, and tingling, mainly in her legs. She had trouble with daily activities such as opening bottles, brushing her hair, and walking. She also had weight loss and shortness of breath with activity. Laboratory studies showed peripheral eosinophilia, elevated inflammatory markers, and thrombocytosis, while CK and aldolase were normal. ANA and scleroderma antibodies were negative, which helped distinguish EF from scleroderma.<br /><br />Physical exam showed hyperpigmentation, skin thickening, peau d’orange changes, and the characteristic “groove sign.” MRI showed abnormal signal in the subcutaneous tissues and muscles, though it did not clearly show fascial thickening. A full-thickness biopsy of the thigh confirmed EF by showing inflammatory cell infiltration.<br /><br />Treatment usually begins with corticosteroids, and methotrexate is the main steroid-sparing agent. This patient received IV methylprednisolone, a steroid taper, and methotrexate, but her disease progressed and required infliximab and rituximab. Her course was complicated by posterior reversible encephalopathy syndrome, adrenal insufficiency, and lumbar compression fractures. She is currently treated with methylprednisolone, methotrexate, and mycophenolate mofetil, along with physical therapy.<br /><br />Key clinical lessons: consider EF in patients with skin tightening, especially when scleroderma antibodies are negative and visceral involvement is limited. Distinctive findings include the groove sign and peau d’orange changes. Most patients respond to steroids, and many improve over 3 to 5 years, though pediatric cases may be more aggressive.

Asset Subtitle

Tina Phan

Meta Tag

Author List Tina Phan

Category Clinical Vignettes

Concept Eosinophilic Fasciitis

Concept Skin Tightness

Concept Diffuse Systemic Sclerosis

Concept Full-Thickness Biopsy

Concept Corticosteroid

Distinguished Non-Finalist

Presenter Organization University of Tennessee Health Science Center

Presenting Author Tina Phan

Track Pediatric

Keywords

eosinophilic fasciitis

pediatric connective tissue disease

scleroderma mimicker

skin tightening

groove sign

peau d’orange

peripheral eosinophilia

methotrexate

corticosteroids

full-thickness biopsy

Eosinophilic Fasciitis

Skin Tightness

Diffuse Systemic Sclerosis

Full-Thickness Biopsy

Corticosteroid