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Eosinophilic Fasciitis (Shulman Syndrome)

Connective tissue disease with peripheral eosinophilia: prune skin and groove sign

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Eosinophilic Fasciitis (Shulman Syndrome)?

Eosinophilic fasciitis is a rare scleroderma-like syndrome described by Shulman in 1974, with female:male ratio of 1:1 and peak between 30-60 years of age. Strenuous exercise (40%), trauma, infection, or drug exposure may precipitate. Annual incidence is 1-2 per million.

Pathophysiology: deep dermis, subcutaneous, fascia, and even muscle layers are infiltrated by eosinophil-rich inflammatory cells, leading to fascial thickening and fibrosis. Hands and feet are typically spared; this distinguishes it from systemic scleroderma.

Diagnosis: peripheral eosinophilia (>20% in 60-80%), polyclonal hypergammaglobulinemia, elevated ESR. Full-thickness skin-muscle biopsy (gold standard) shows fascial thickening, eosinophil infiltration. MRI: T2 hyperintensity, fascial thickening, gadolinium enhancement. Steroid response is usually rapid and dramatic.

Symptoms

Sudden onset symmetric upper-lower extremity edema
Diffuse induration and prune-skin appearance
Groove sign (linear depression along veins on elevation)
Sparing of fingers (distinguishes from scleroderma)
Joint contracture (especially hands, elbows)
Carpal tunnel syndrome and arthralgia

Risk Factors

Strenuous physical exercise (40% precipitating)
Trauma or surgery history
Borrelia burgdorferi exposure
Hematologic malignancy (10-15%)
Drug (statin, simvastatin, ramipril, etc.)
Adult age (30-60 years)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Sudden onset symmetric extremity edema
  • Skin induration and prune-skin appearance
  • Joint contracture and reduced movement
  • Peripheral eosinophilia (>1.5 × 10⁹/L)
  • Carpal tunnel symptoms and arthralgia
  • Lesions developing rapidly after exercise/trauma

Treatment Methods

01
High-dose prednisolone (1 mg/kg/day) - first line
02
Methotrexate (15-25 mg/week) - steroid sparing/resistance
03
Pulse methylprednisolone (severe, refractory)
04
Mycophenolate mofetil (MTX-resistant)
05
Tocilizumab, rituximab (refractory)
06
Physiotherapy (contracture prevention)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.