Dermatologic Manifestations of Eosinophilic Fasciitis

Updated: Jan 31, 2017
  • Author: Brad S Graham, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Eosinophilic fasciitis is an idiopathic, fibrotic disorder with the histopathologic hallmark of fascial fibrosis. The presentation of eosinophilic fasciitis is acute with painful, swollen extremities progressing to disabling cutaneous fibrosis. Joint contractures, arthritis, neuropathy, and myositis may be associated with eosinophilic fasciitis. Many authors consider eosinophilic fasciitis to be a variant of morphea; others consider it a distinct entity.

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Pathophysiology

 

The etiology of eosinophilic fasciitis is unknown, but aberrant immune responses may play a role because hypergammaglobulinemia and antinuclear antibodies are associated. In addition, toxic, environmental, or drug exposures have been implicated in causing eosinophilic fasciitis. A 2006 case report implicated atorvastatin in a temporal relationship as the cause of a patient's eosinophilic fasciitis. [1] Simvastatin has also been reported temporally to the onset of eosinophilic fasciitis. [2]

Reports indicate that Borrelia burgdorferi may be a possible etiologic agent in some cases of eosinophilic fasciitis. However, one report of a patient with eosinophilic fasciitis and a review of the literature of cases in which Borrelia species were implicated in the pathogenesis failed to show a relationship between eosinophilic fasciitis and Borrelia infection. Borrelia species were not identified by direct microscopic examination of tissue samples or by polymerase chain reaction amplification of tissue samples in any of these reported cases of eosinophilic fasciitis. The conclusion was that positive serology alone for Borrelia does not implicate Borrelia infection in the pathogenesis of eosinophilic fasciitis in the absence of the positive demonstration of Borrelia by histochemical stains, immunohistochemical stains, or polymerase chain reaction amplification in tissue samples. [3]

In vitro fibroblasts from involved fascia produce increased levels of mRNA for collagen types I, III, and IV compared with adjacent dermal fibroblasts. In addition, fascial fibroblasts express transforming growth factor-beta I and connective-tissue growth factor mRNA, which may account for the clinical fibrosis. Eosinophil degranulation may lead to fibroblast activation.

Further research into eosinophilic fasciitis has shown elevations of transforming growth factor-beta and interleukin 5, which normalize with corticosteroid therapy. Another study has show that the fascial inflammatory infiltrate is predominately composed of CD8+ T lymphocytes, macrophages, and fewer eosinophils, suggesting a possible cytotoxic immune reaction in response to possible infectious or environmental agents. Other studies have shown elevated serum levels of manganese superoxide dismutase and tissue inhibitor of metalloproteinase (TIMP-1). Serum TIMP-1 may also serve as a marker of disease severity. [4, 5]

One report describe of diffuse eosinophilic fasciitis developing after local radiation therapy for breast cancer, implicating radiation injury as a possible traumatic trigger for the development of eosinophilic fasciitis. [6]

An additional case report in 2010 identified a patient with a temporal and anatomic relationship with the development of localized eosinophilic fasciitis after intravenous iron infusions for correction of anemia. [7]

A 2012 case report describes of a young male with eosinophilic fasciitis, recurrent fever, a sclerotic prepuce, and urethritis. Urethral and blood cultures were positive for Mycoplasma arginini. In addition, M arginini DNA was isolated in skin biopsy specimens of sclerotic skin by polymerase chain reaction (PCR). The authors suggested that the Mycoplasma infection may have contributed to the development of the sclerotic process. [8]

A case report in 2016 discussed a male patient with the development of eosinophilic fasciitis following an intestinal parasitic infection. Treatment with antiparasitic drugs, however, did not lead to resolution of symptoms. [9]

 

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Epidemiology

Frequency

Eosinophilic fasciitis is uncommon.

Race

Whites are primarily affected by eosinophilic fasciitis.

Sex

Eosinophilic fasciitis occurs equally in males and females.

Age

Most eosinophilic fasciitis patients are in their third to sixth decades of life; however, cases in children have been reported.

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Prognosis

The prognosis for eosinophilic fasciitis is good. Most patients experience partial or complete recovery.

The end stage of the fibrotic process leads to substantial morbidity due to skin sclerosis and joint contractures. In addition, arthritis, neuropathies, and myositis may be present. Ten percent of cases may result in myelodysplasia, such as aplastic anemia, which portends a poor prognosis. Spontaneous resolution is possible, and treatment with corticosteroids usually results in recovery; however, skin sclerosis and joint contractures may persist.

A 2007 study reported that the risk of residual fibrosis/contractures after therapy was much higher with an age younger than 12 years at presentation, trunk involvement, associated morphealike, and dermal fibrosis in addition to the subcutaneous fat/fascial fibrosis. [10]

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