Eosinophilic Fasciitis Differential Diagnoses

Updated: Dec 06, 2018
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
  • Print
DDx

Diagnostic Considerations

Differential considerations include the following:

  • The localized forms of scleroderma, morphea, and linear forms of scleroderma

  • Limited and diffuse cutaneous systemic sclerosis

  • Other localized cutaneous fibrosing disorders, eg, nephrogenic systemic fibrosis, scleromyxedema, and scleredema

In general, these alternative etiologies can be excluded by the absence of peripheral eosinophilia. However, cases of diffuse morphea with features that overlap with eosinophilic fasciitis have been reported. [36, 37] The absences of Raynaud phenomenon, abnormal capillaroscopy findings, and visceral involvement are key findings that differentiate eosinophilic fasciitis (EF) from systemic sclerosis.

Eosinophilia-myalgia syndrome and toxic oil syndrome are two disorders that share common clinical and histopathological features with eosinophilic fasciitis, including peripheral eosinophilia. In contrast to eosinophilic fasciitis, these two conditions present in epidemic form and, after epidemiological analysis, appear to be almost universally toxin-associated. [38, 39]

In 1989, an epidemic of a connective-tissue disease with peripheral eosinophilia and prominent myalgias was recognized in the United States and was therefore termed eosinophilic myalgia syndrome. Subsequent epidemiologic studies indicated that most individuals who developed eosinophilic myalgia syndrome had consumed l-tryptophan from a single source. Diffuse induration of the integument affecting the extremities and occasionally the torso, but sparing the face, hands, and feet, developed in a large number of patients with eosinophilic myalgia syndrome. In contrast to eosinophilic fasciitis, patients with eosinophilic myalgia syndrome typically presented with prominent systemic symptoms, including fever, myalgia, and rash.

Similarly, in 1980 in Spain, an epidemic of connective-tissue disease with peripheral eosinophilia was recognized. The epidemic was eventually traced to rapeseed oil that had been denatured with aniline and sold in bulk as olive oil for cooking. As many as 20,000 Spaniards were affected. Acute symptoms were similar to those of eosinophilic myalgia syndrome, with prominent systemic symptoms in addition to cutaneous changes. In contrast to eosinophilic myalgia syndrome, acute symptoms included more prominent pulmonary manifestations. In chronic disease, patients with toxic oil syndrome were more likely to demonstrate systemic (pulmonary, neurologic) symptoms. [12, 40, 41]

Differential Diagnoses