What are extracutaneous manifestations of eosinophilic fasciitis (EF)?

Updated: Dec 24, 2020
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Extracutaneous manifestations include the following:

  • Joint contractures represent the most common extracutaneous manifestation of eosinophilic fasciitis, occurring in 50%-75% of patients, and can affect elbows, wrists, ankles, knees, and shoulders. [14, 15]  Extensive truncal fibrosis may limit chest expansion. A clawlike deformity of the hand has been described.
  • Inflammatory arthritis was reported in roughly 40% of patients in two series. [14, 15]  The knees, wrists, hands, and feet appear to be most commonly involved.
  • Carpal tunnel syndrome is seen in 16%-23% of patients. [14, 15, 36]
  • Clinically significant visceral involvement is rare, limited to case reports. If present, significant visceral involvement should prompt investigation of an alternative diagnosis. When pursued, specific testing with pulmonary function testing, esophagogastroduodenoscopy (EGD), and electromyelography (EMG) may demonstrate subtle or nonspecific abnormalities. [37]

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