Rapid onset woody induration of the extremities in association with peripheral eosinophilia
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Clinical presentation
- Strenuous physical activity precedes in 30%
- Edema and pain of involved extremities
- → sclerosis, dimple, “pseudo-celullitis” appearance
- Symmetric distribution
- “Groove sign” - linear depressions where veins appear to be sunken within indurated skin
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Labs
- Elevated ESR
- Hypergammaglobulinemia
- Peripheral eosinophilia
- ANA normal
- Complement normal
- Hypereosinophilia work-up → see hypereosinophilic syndromes
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Histology
- Thickened fascia
- Eosinophilic infiltrate
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Diagnosis
- Histology and/or MRI
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Treatment
- Corticosteroids
- Prednisolone 1-2mg/kg id
- Tapered over 6-24 months
- MTX
- MMF
- IVIg
- Hydroxichloroquine
- JAK inhibitors
- UVA-phototherapy
- Cyclosporine
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