eMedicine Specialties > Dermatology > Connective Tissue Diseases
Eosinophilic Fasciitis
Updated: Jun 19, 2009
Introduction
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.
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.2Reports 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
Frequency
International
Eosinophilic fasciitis is uncommon.
Mortality/Morbidity
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.7
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.
Clinical
History
- Eosinophilic fasciitis patients present with the sudden onset of painful, tender, edematous, and erythematous extremities.
- The disorder progresses rapidly; within weeks to months, patients develop stiffness and sclerodermatous induration, resulting in characteristic flexion contractures and impaired mobility.
- The forearms, the upper arms, the lower legs, the thighs, and the trunk are involved (in order of decreasing frequency).
- As many as 50% of patients report an episode of strenuous physical exercise or activity immediately preceding the onset of the illness.
- Malaise, weakness, and fever are frequently present. A study in 2008 addressing the physical burden noted that fatigue was the most common symptom. Pain and itch were also frequent complaints. Of all patients, 62% noted fatigue, pain, and itch.8
- Overt arthritis occurs in as many as 40% of patients.
- Symptoms of carpal tunnel syndrome have been reported.
- Visceral involvement and Raynaud phenomenon are rare.
Physical
The clinical presentation of eosinophilic fasciitis evolves through 3 stages; the various stages present simultaneously in different areas of the body. The first stage presents with symmetric, diffuse, erythematous tenderness of the extremities, followed by an edematous phase that produces a coarsely dimpled appearance (cobblestoning) or a finely dimpled appearance (peau d'orange). The last phase involves rippling of the skin with areas of hypopigmentation, induration, and skin tightness.
Lower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement (same patient as in Media Files 2-3).
Posterior thigh shows woody induration, sclerosis, and hypopigmentation (same patient as in Media Files 1 and 3).
Close-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration (same patient as in Media Files 1-2).
Posterior part of the calf in the first week of illness shows erythema, edema, alopecia, scaling, and early induration. The right calf is relatively uninvolved with patchy erythema only (same patient as in Media File 5).
Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema (same patient as in Media File 4).
In severely affected areas, both the skin and the subcutaneous tissues are bound-down and inseparable from the underlying muscle, and they have a woody-type appearance. With elevation of the involved extremities, furrows along the course of the superficial veins may be present; this finding is referred to as the groove sign. Although the extremities are preferentially involved (88%), the trunk may be involved. The hands, the feet, and the face are spared.
- Joint contractures of the elbows, wrists, ankles, knees, and shoulders may be found in 55-75% of patients. Unilateral involvement has been reported.9
- Carpal tunnel syndrome is present in 20% of patients.
- Inflammatory arthritis is present in as many as 40% of patients.
- Subclinical myositis is present in a minority of patients.
- A concurrent localized lesion of morphea may be seen in 25% of patients.
- In contrast to scleroderma, Raynaud phenomenon, abnormal nail fold capillaries, and sclerodactyly are not present. Visceral involvement is rare, with few reports of involvement of the lungs, the esophagus, and the myocardium.
- Primary presentation as angioedema was also reported in a patient.10
Causes
See Pathophysiology.
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| References |
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References
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Further Reading
Keywords
eosinophilic fasciitis, Shulman syndrome, morphea, fascial fibrosis, joint contractures, arthritis, neuropathy, myositis
