Dermatologic Manifestations of Eosinophilic Fasciitis 

  • Author: Brad S Graham, MD; Chief Editor: William D James, MD   more...
 
Updated: Aug 16, 2011
 

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]

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Epidemiology

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.[8]

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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Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Brad S Graham, MD  Consulting Staff, Dermatology Associates of Tyler

Brad S Graham, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

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  2. Serrano-Grau P, Mascaró-Galy JM, Iranzo P. Eosinophilic fasciitis after taking simvastatin. Actas Dermosifiliogr. Jun 2008;99(5):420-1. [Full Text].

  3. Anton E. Failure to demonstrate Borrelia burgdorferi-specific DNA in lesions of eosinophilic fasciitis. Histopathology. Jul 2006;49(1):88-90. [Medline].

  4. Jinnin M, Ihn H, Yazawa N, Asano Y, Yamane K, Tamaki K. Elevated serum levels of manganese superoxide dismutase in patients with eosinophilic fasciitis. Clin Rheumatol. Dec 2003;22(6):505. [Medline].

  5. Jinnin M, Ihn H, Yamane K, Asano Y, Yazawa N, Tamaki K. Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis. Br J Dermatol. Aug 2004;151(2):407-12. [Medline].

  6. Sherber NS, Wigley FM, Paget SA. Diffuse fasciitis with eosinophilia developing after local irradiation for breast cancer. Clin Rheumatol. Jun 2009;28(6):729-32. [Medline].

  7. Firoz BF, Goldberg LH, Landau J, Kaye V, Berman L. Eosinophilic fasciitis secondary to intravenous iron infusions. Dermatol Online J. May 15 2010;16(5):5. [Medline].

  8. Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol. Sep 2007;26(9):1445-51. [Medline].

  9. Kroft EB, de Jong EM, Evers AW. Physical burden of symptoms in patients with localized scleroderma and eosinophilic fasciitis. Arch Dermatol. Oct 2008;144(10):1394-5.

  10. Daniel RS, Brown AN. Case report of unilateral eosinophilic fasciitis in a Vietnamese woman. Am J Med Sci. Feb 2009;337(2):153-4. [Medline].

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  12. Varga J, Griffin R, Newman JH, Jimenez SA. Eosinophilic fasciitis is clinically distinguishable from the eosinophilia-myalgia syndrome and is not associated with L-tryptophan use. J Rheumatol. Feb 1991;18(2):259-63. [Medline].

  13. Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. Nov 1997;9(6):562-70. [Medline].

  14. Baumann F, Bruhlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. Jan 2005;184(1):169-74. [Medline].

  15. Valencia IC, Chang A, Kirsner RS, Kerdel FA. Eosinophilic fasciitis responsive to treatment with pulsed steroids and cyclosporine. Int J Dermatol. May 1999;38(5):369-72. [Medline].

  16. Romano C, Rubegni P, De Aloe G, et al. Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol. Jan 2003;17(1):10-3. [Medline].

  17. Tzaribachev N, Holzer U, Schedel J, Maier V, Klein R, Kuemmerle-Deschner J. Infliximab effective in steroid-dependent juvenile eosinophilic fasciitis. Rheumatology (Oxford). Jun 2008;47(6):930-2. [Medline].

  18. Haiduc VF, Erkan D, Kirou K, Birchansky S, Park J, Danon MJ. Anti-neutrophil cytoplasmic antibody (c-ANCA) positive recurrent eosinophilic fasciitis responsive to cyclophosphamide: a clinical pathology conference held by the Division of Rheumatology at Hospital for Special Surgery. HSS J. Feb 2008;4(1):81-6. [Medline]. [Full Text].

  19. Smith LC, Cox NH. Dapsone treatment for eosinophilic fasciitis. Arch Dermatol. Jul 2008;144(7):845-7. [Medline].

  20. Weber HO, Schaller M, Metzler G, Röcken M, Berneburg M. Eosinophilic fasciitis and combined UVA1--retinoid--corticosteroid treatment: two case reports. Acta Derm Venereol. 2008;88(3):304-6. [Medline].

  21. Horacek E, Sator PG, Gschnait F. 'Venous furrowing': a clue to the diagnosis of eosinophilic fasciitis. A case of eosinophilic fasciitis ultimately treated with oral PUVA therapy. Dermatology. 2007;215(1):89-90. [Medline].

  22. Khanna D, Agrawal H, Clements PJ. Infliximab may be effective in the treatment of steroid-resistant eosinophilic fasciitis: report of three cases. Rheumatology (Oxford). Jun 2010;49(6):1184-8. [Medline]. [Full Text].

  23. Pimenta S, Bernardes M, Bernardo A, Brito I, Castro L, Simões-Ventura F. Intravenous immune globulins to treat eosinophilic fasciitis: a case report. Joint Bone Spine. Oct 2009;76(5):572-4. [Medline].

  24. Kitamura Y, Hatamochi A, Hamasaki Y, Ikeda H, Yamazaki S. Association between eosinophilic fasciitis and systemic lupus erythematosus. J Dermatol. Feb 2007;34(2):150-2. [Medline].

  25. Khalifa M, Slim I, Ghannouchi N, et al. Eosinophilic fasciitis and toxic thyroid adenoma--a novel association. Acta Clin Belg. May-Jun 2008;63(3):197-9.

  26. Tahara K, Yukawa S, Shoji A, Hayashi H, Tsuboi N. Long-term remission by cyclosporine in a patient with eosinophilic fasciitis associated with primary biliary cirrhosis. Clin Rheumatol. Sep 2008;27(9):1199-201. [Medline].

 
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Lower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement.
Posterior thigh shows woody induration, sclerosis, and hypopigmentation.
Close-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration.
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.
Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema.
Note the marked thickening and replacement of the entire dermis with sclerotic collagen on this incisional biopsy sample from the left posterior part of the thigh.
Photomicrograph of subcutaneous fat-fascia junction shows entrapment of subcutaneous fat by intersecting thick bands of fibrosis. Thickening and fibrosis of fascia and lymphoid aggregates are seen.
Photomicrograph of fascia-skeletal muscle junction shows markedly thickened fascia with heavy inflammatory infiltration.
High-power photomicrograph of fascia shows heavy inflammatory infiltration with numerous eosinophils, lymphocytes, and occasional plasma cells.
 
 
 
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