Eosinophilic fasciitis (Schulmann's syndrome) (ICD-10 M35.4)

Definition

Eosinophilic fasciitis is a rare disease that attacks the subcutaneous and fascia surrounding the muscles without any known cause.

Occurrence

• Men (40-50 years) are attacked a little more frequently than women
• The disease is described in children, but extremely rare
• Eosinophilic fasciitis is defined as one rare disease

Symptoms

• Disease debut
  • Symptoms after physical activity, then gradual development
  • Pain
• Tight and tender, hot skin
• Swelling on the inside of the arms and legs
• Symmetrical changes (approximately equal on both sides of the body) are most common
  • Skin of the face, chest and stomach area may be attacked
  • No on fingers and toes (as opposed to Systemic sclerosis)
  • No Raynaud's phenomenon  (Unlike Systemic sclerosis)
  • Inner organs are not attacked
• After weeks, the skin becomes inflamed and hard, partly with orange-peel-like indentations. Reduced mobility in joints (elbows, shoulders, hips, knees) may occur. Squeezing nerves in hands (Carpal tunnel syndrome) may also occur

Medical examinations

Blood tests  

• Increased Erythrocyte sedimentation rate (ESR), CRP and eosinophilic white blood cells. Some may slightly raised CK when at the same time muscle manifestation occur. No special Antibodies

Bone marrow examination

• Approximately 10% with eosinophilic fasciitis also have a blood disease (various types). In case of suspicion of blood disease, further investigation with bone marrow examination is relevant

Tissue sample (biopsy)

• Biopsy shows eosinophilia in tissues. Biopsy is taken through all skin layers down to muscle fascia

MR

• MRI (with contrast agent or STIR) shows inflammation (edema) at the muscle fascia (see illustration above)

Incorrect diagnosis (similar conditions, differential diagnoses)

• Systemic sclerosis

  • Raynaud's
  • Fingers have skin changes

• Graft vs. Host Disease (GVHD)

  • Immune system responds to foreign matter

• Linear scleroderma / morphea

  • Most common among children, but can begin in adulthood
  • Mainly, one side of the body is attacked

• Nephrogenic systemic fibrosis

  • After MR contrast in renal failure

• Skleredema (Buschke)

• Eosinophilia-myalgia syndrome

  • After ingestion of L-tryptophan

• Toxic cooking oil syndrome

  • Occured in Spain 1981

Treatment

• Corticosteroids (Prednisone)
  • High doses to prevent scarring and persistent reduced mobility. Early treatment is most beneficial for symptoms and for the course of the disease. Rapid response to inflammation, but slow recovery. Gradual phasing out of treatment over 2-5 years
  • About half with eosinophilic fasciitis need other immunosuppressive therapy as well (please see below)
• Methotrexate
• Plaquenil
• Sandimune 

Medical prognosis / Life expectancy

• Gradual improvement after a few years. Many become almost completely symptom-free in the long run. The disease is unlikely to affect life expectancy.

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