EOSINOPHILIC FASCIITIS

Overview and Facts

Eosinophilic fasciitis (EF) was first described in 1974 as a swelling of the connective tissue overlying the different groups of muscles as a result of the strain brought about by the excess activity during exercise. It also goes by the name Shulman’s syndrome and is identified to have presentations similar to that of scleroderma. While scleroderma affects the skin, EF affects the thin sheath covering the muscles, blood vessels, and nerves.

Eosinophilic fasciitis is unique in that it affects the extremities only and is associated with a specific type of anemia where there is a deficient production of all cell lines, aplastic anemia. The cause is unknown, but several studies have shown that it is closely related to the reaction of the body to an allergic stimulus, and the result would be the mounting of the inflammatory response of the body resulting in this condition.

Types and Symptoms

SYMPTOMS

Pain and a feeling of tiredness, especially on the affected extremity (arms, forearms, legs, and thighs), are the most common symptom of Eosinophilic fasciitis. It can also present with any of the following:

  • Tenderness over the affected muscle group
  • Formation of a venous track, there is a depression in the area following the course of the vein.
  • Redness of the overlying skin
  • Warm to touch
  • There may be the presence or appearance of thickening and hardening of the skin texture
  • In more severe cases, it is noted that a formation of irregular skin dimpling resembling that of an orange peel.
  • Limitation of motion of the affected extremity causing a permanent inability to extend the affected extremity, e.g., frozen shoulder syndrome
  • Other non-specific signs and symptoms such as low-grade fever, loss of appetite, a general feeling of tiredness, inability to bear weight on the affected extremity,
  • Arthritic like symptoms such as pain at the joints on the mobilization of the extremities
  • In some severe cases, pain on the bones is reported.
  • Numbness and tingling of the affected extremities
  • Inability to clasp objects by hand

Diagnosis & Medications

DIAGNOSIS

Thorough history followed by physical examination can alert the suspicion of eosinophilic fasciitis. The clinician usually notes the presence of tender extremities on deep palpation on a febrile patient can point to the diagnosis of EF.

Laboratory tests include the following:

  • Complete blood count- there is an increase in the number of white blood cells that signifies inflammation.
  • Erythrocyte sedimentation rate (ESR)- measures the rate at which the red blood cells that settle in the tube. An elevated ESR signifies infection.
  • Biopsy of the involved muscle- this is considered as the gold standard of diagnosis. This is done by extraction of the affected tissue through surgery and subsequent examination under the microscope to identify the presence of inflammation.

TREATMENT

The goal of treatment is to prevent the overwhelming response of the body to the inciting agent. Several treatment options include:

  • High dose steroid treatment- prednisone or methylprednisone administered orally can cause inflammation to subside
  • Surgical debridement- This is done in cases where there is an accumulation of pus in the fascia. This typically provides instantaneous relief.

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