Eosinophilic fasciits

Eosinophilic fasciitis is a rare scleroderma-like condition characterised by an acute presentation of pain, swelling and tenderness of the distal limbs, progressing to fibrosis, which can result in reduced mobility of the hands, feet and other joints.

This chapter is set out as follows:  

• The cause is unknown

• Eosinophilic fasciitis predominantly occurs in Caucasians, with females and males equally affected
• It may occur at any age, although most cases arise between the ages of 30 and 60 years
• It has an acute, painful onset 
• Malaise, weakness, and fever are frequently present

• There are three cutaneous stages
  • The initial phase is a symmetric, diffuse, erythematous tenderness of the distal limbs. The trunk may be involved, but the hands, feet, and the face are spared
  • The skin then becomes oedematous with a characteristic orange-peel appearance
  • The final phase involves fibrotic change with areas of hypopigmentation, induration, and skin tightness of the ankles and wrists 
     
• In severely affected cases both the skin and subcutaneous tissues are bound-down to the underlying muscle, with a woody-like appearance. In such cases joint contractures can be more extensive and involve the knees, elbows, and shoulders 
   
• Other clinical features sometimes reported include the Carpal Tunnel syndrome, and inflammatory arthritis

• Differential 
  • Pansclerotic morphoea has a more insidious onset and is characterised by near total body surface involvement, with circumferential lesions, sparing the fingers and toes, which usually extends into the subcutaneous tissue, and may affect the fascia, muscle, and bone
  • Unlike in systemic sclerosis, Raynaud's phenomenon, abnormal nailfold capillaries, and sclerodactyly are absent
    

• A FBC reveals eosinophilia in 70% of cases. Some patients have an elevated ESR and hyperglobulinaemia

• A full-thickness skin biopsy that includes the dermis, subcutaneous fat and fascia is necessary to confirm a diagnosis of eosinophilic fasciitis, and shows dermal sclerosis, with inflammation and fibrosis of the fat and deep fascia. The fascia is thickened and infiltrated with several cell types including lymphocytes and eosinophils (reference: Rook's Textbook of Dermatology)

• If clinically indicated, MRI of the involved areas shows a high-intensity signal in the fascia

• Oral corticosteroids are the mainstay of treatment, with most patients responding well to moderate-to-high doses, particularly if started early in the course of the condition. Maintenance low-dose treatment may be required for 2-5 years

• Steroid-sparing drugs such as hydroxychloroquine, methotrexate, and ciclosporin are sometimes required. More recently there has been success with the biologics 

• Physiotherapy is required to prevent and treat joint contractures

• In some cases the disease resolves spontaneously