eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease

Eosinophilic Fasciitis: Treatment & Medication

Author: Peter M Henning, DO, MAJ, MC, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Coauthor(s): George R Mount, MD, MAJ USA MC, Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Attending Physician, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC; Nicholas David Kortan, DO, CPT, MC,, Resident Physician, Department of Internal Medicine, Walter Reed Army Medical Center, Washington, DC; Sherif Nasef, MD, Consulting Staff, Department of Internal Medicine, Division of Rheumatology, Lake Havasu Regional Medical Center; Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Contributor Information and Disclosures

Updated: Oct 2, 2009

Treatment

Medical Care

When considering medical therapies for eosinophilic fasciitis (EF), especially second-line agents, it should be noted that up to one third of eosinophilic fasciitis cases may spontaneously resolve.

  • Initial therapy15,16,17,36,46,50
    • There is wide consensus that systemic corticosteroids are the initial therapeutic agent of choice. Typically, prednisone or prednisolone is used, in doses ranging from 20-100 mg/day.
    • Response is considered satisfactory with reduction in edema, improvement in skin thickening, resolution of carpal tunnel syndrome, and gradual decrease in joint contracture.
    • Eosinophilia and inflammatory markers frequently resolve promptly after initiation of prednisone therapy.
  • Additional therapeutic agents and adjunctive therapies16,51,52,53,54
    • Multiple additional therapeutic agents have been used as disease-modifying or steroid-sparing agents in persistent or steroid-resistant cases of eosinophilic fasciitis. It should be noted that there is no general consensus with regard to the best agent for this type of disease. Treatment numbers are generally small, and controlled trials are lacking.
    • Case reports detail the use of multiple additional agents, including antihistamines, cimetidine, hydroxychloroquine, chloroquine, azathioprine, cyclosporine, dapsone, infliximab, tacrolimus, methotrexate, D-penicillamine, griseofulvin, ketotifen, and alpha-interferon, with varying rates of response. Some recent data suggest that other anti–tumor necrosis factor (TNF)–alpha agents may also be beneficial.
    • Physical therapy should be initiated to improve joint mobility and to decrease contractures.
    • Surgical release has been used in some cases to manage significant joint contractures.55

Surgical Care

Surgical release has also been used in some cases of eosinophilic fasciitis for significant joint contractures.55

Consultations

Dermatologists, rheumatologists, and surgeons (for the skin-muscle biopsy) are consulted most often.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


Prednisone (Sterapred)

Useful in the treatment of inflammatory conditions by reversing increased capillary permeability and suppressing neutrophil activity.

Adult

20-60 mg/d PO usually undivided

Pediatric

4-5 mg/m2/d PO

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

No absolute contraindications; documented hypersensitivity; severe bacterial, viral, or fungal infection; active peptic ulcer disease; diabetes mellitus

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use; abrupt discontinuation of glucocorticoids may cause adrenal crisis

More on Eosinophilic Fasciitis

Overview: Eosinophilic Fasciitis
Differential Diagnoses & Workup: Eosinophilic Fasciitis
Treatment & Medication: Eosinophilic Fasciitis
Follow-up: Eosinophilic Fasciitis
Multimedia: Eosinophilic Fasciitis
References
[ CLOSE WINDOW ]

References

  1. Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome?. Trans Assoc Am Physicians. 1975;88:70-86. [Medline].

  2. 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].

  3. Viallard JF, Taupin JL, Ranchin V, Leng B, Pellegrin JL, Moreau JF. Analysis of leukemia inhibitory factor, type 1 and type 2 cytokine production in patients with eosinophilic fasciitis. J Rheumatol. Jan 2001;28(1):75-80. [Medline].

  4. Dziadzio L, Kelly EA, Panzer SE, Jarjour N, Huttenlocher A. Cytokine abnormalities in a patient with eosinophilic fasciitis. Ann Allergy Asthma Immunol. Apr 2003;90(4):452-5. [Medline].

  5. Toquet C, Hamidou MA, Renaudin K, Jarry A, Foulc P, Barbarot S. In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis. J Rheumatol. Aug 2003;30(8):1811-5. [Medline].

  6. Kahari VM, Heino J, Niskanen L, et al. Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol. May 1990;126(5):613-7. [Medline].

  7. Peltonen J, Kahari L, Jaakkola S, et al. Evaluation of transforming growth factor beta and type I procollagen gene expression in fibrotic skin diseases by in situ hybridization. J Invest Dermatol. Mar 1990;94(3):365-71. [Medline].

  8. Mori Y, Kahari VM, Varga J. Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep. Apr 2002;4(2):113-22. [Medline].

  9. 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].

  10. Moutsopoulos HM, Webber BL, Pavlidis NA, Fostiropoulos G, Goules D, Shulman LE. Diffuse fasciitis with eosinophilia. A clinicopathologic study. Am J Med. May 1980;68(5):701-9. [Medline].

  11. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. Aug 1979;96(2):493-518. [Medline].

  12. Brent LH, Abruzzo JL. Localized eosinophilic fasciitis in a patient with rheumatoid arthritis. J Rheumatol. Oct 1985;12(5):987-9. [Medline].

  13. Allen SC. Eosinophilic fasciitis in an African--possible benefit of chloroquine treatment. Postgrad Med J. Oct 1984;60(708):685-6. [Medline].

  14. Nawata Y, Sueishi M, Koike T, Tomioka H. Eosinophilic fasciitis with autoimmune features. Arthritis Rheum. May 1983;26(5):688. [Medline].

  15. Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. May 1988;17(4):221-31. [Medline].

  16. Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature. Dermatology. 2006;213(2):93-101. [Medline].

  17. Bischoff L, Derk CT. Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature. Int J Dermatol. Jan 2008;47(1):29-35. [Medline].

  18. Wollheim FA, Lindstrom CG, Eiken O. Eosinophilic fasciitis complicated by carpal tunnel syndrome. J Rheumatol. Sep-Oct 1981;8(5):856-60. [Medline].

  19. Caspi D, Fishel R, Varon M, et al. Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil. Nov 1982;21(4):218-21. [Medline].

  20. Choquet-Kastylevsky G, Kanitakis J, Dumas V, Descotes J, Faure M, Claudy A. Eosinophilic fasciitis and simvastatin. Arch Intern Med. Jun 11 2001;161(11):1456-7. [Medline].

  21. DeGiovanni C, Chard M, Woollons A. Eosinophilic fasciitis secondary to treatment with atorvastatin. Clin Exp Dermatol. Jan 2006;31(1):131-2. [Medline].

  22. Buchanan RR, Gordon DA, Muckle TJ, McKenna F, Kraag G. The eosinophilic fasciitis syndrome after phenytoin (dilantin) therapy. J Rheumatol. Sep-Oct 1980;7(5):733-6. [Medline].

  23. Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol. Oct 1996;18(5):465-73. [Medline].

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

  25. Blauvelt A, Falanga V. Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination. Arch Dermatol. Aug 1991;127(8):1159-66. [Medline].

  26. Hibbs JR, Mittleman B, Hill P, Medsger TA Jr. L-tryptophan-associated eosinophilic fasciitis prior to the 1989 eosinophilia-myalgia syndrome outbreak. Arthritis Rheum. Mar 1992;35(3):299-303. [Medline].

  27. Lee P. Eosinophilic fasciitis: new associations and current perspectives [editorial]. J Rheumatol. Jan-Feb 1981;8(1):6-8. [Medline].

  28. Doyle JA, Connolly SM, Hoagland HC. Hematologic disease in scleroderma syndromes. Acta Derm Venereol. 1985;65(6):521-5. [Medline].

  29. Masuoka H, Kikuchi K, Takahashi S, Kakinuma T, Hayashi N, Furue M. Eosinophilic fasciitis associated with low-grade T-cell lymphoma. Br J Dermatol. Nov 1998;139(5):928-30. [Medline].

  30. Garcia VP, de Quiros JF, Caminal L. Autoimmune hemolytic anemia associated with eosinophilic fasciitis. J Rheumatol. Sep 1998;25(9):1864-5. [Medline].

  31. Hur JW, Lee HS, Uhm WS, et al. Eosinophilic fasciitis associated with autoimmune thyroiditis. Korean J Intern Med. Jun 2005;20(2):180-2. [Medline].

  32. Katz JD, Wakem CJ, Parke AL. L-tryptophan associated eosinophilia-myalgia syndrome. J Rheumatol. Nov 1990;17(11):1559-61. [Medline].

  33. Shulman LE. The eosinophilia-myalgia syndrome associated with ingestion of L- tryptophan. Arthritis Rheum. Jul 1990;33(7):913-7. [Medline].

  34. Kaufman LD, Krupp LB. Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia. Curr Opin Rheumatol. Nov 1995;7(6):560-7. [Medline].

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

  36. Abeles M, Belin DC, Zurier RB. Eosinophilic fasciitis: a clinicopathologic study. Arch Intern Med. May 1979;139(5):586-8. [Medline].

  37. Falanga V, Medsger TA Jr. Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis. J Am Acad Dermatol. Oct 1987;17(4):648-56. [Medline].

  38. Kim SW, Rice L, Champlin R, Udden MM. Aplastic anemia in eosinophilic fasciitis: responses to immunosuppression and marrow transplantation. Haematologia (Budap). 1997;28(3):131-7. [Medline].

  39. Naschitz JE, Yeshurun D, Zuckerman E, Rosenbaum M, Misselevitch I, Shajrawi I. Cancer-associated fasciitis panniculitis. Cancer. Jan 1 1994;73(1):231-5. [Medline].

  40. Sugimoto T, Nitta N, Kashiwagi A. Usefulness of magnetic resonance imaging in eosinophilic fasciitis. Rheumatol Int. Jun 2007;27(8):791-2. [Medline].

  41. Moulton SJ, Kransdorf MJ, Ginsburg WW, Abril A, Persellin S. Eosinophilic fasciitis: spectrum of MRI findings. AJR Am J Roentgenol. Mar 2005;184(3):975-8. [Medline].

  42. Agnew KL, Blunt D, Francis ND, Bunker CB. Magnetic resonance imaging in eosinophilic fasciitis. Clin Exp Dermatol. Jul 2005;30(4):435-6. [Medline].

  43. Baumann F, Bruhlmann P, Andreisek G, et al. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. Jan 2005;184(1):169-74. [Medline].

  44. Dybowski F, Neuen-Jacob E, Braun J. Eosinophilic fasciitis and myositis: use of imaging modalities for diagnosis and monitoring. Ann Rheum Dis. Apr 2008;67(4):572-4. [Medline].

  45. Bertken R, Shaller D. Chronic progressive eosinophilic fasciitis: report of a 20-year failure to attain remission. Ann Rheum Dis. Feb 1983;42(1):103-5. [Medline].

  46. Carneiro S, Brotas A, Lamy F, et al. Eosinophilic fasciitis (Shulman syndrome). Cutis. Apr 2005;75(4):228-32. [Medline].

  47. 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].

  48. Cramer SF, Kent L, Abramowsky C, Moskowitz RW. Eosinophilic fasciitis. Immunopathology, ultrastructure, literature review,a nd consideration of its pathogenesis and relation to scleroderma. Arch Pathol Lab Med. Feb 1982;106(2):85-91. [Medline].

  49. Kähäri VM, Heino J, Niskanen L, Fräki J, Uitto J. Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol. May 1990;126(5):613-7. [Medline].

  50. Chan MK, Lages W. Eosinophilic fasciitis: visceral involvement. Arch Intern Med. Nov 1982;142(12):2201-2. [Medline].

  51. 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].

  52. 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].

  53. Herson S, Brechignac S, Godeau P. Cimetidine in eosinophilic fasciitis. Ann Intern Med. Sep 1 1990;113(5):412-3. [Medline].

  54. Bukiej A, Dropinski J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. Nov 2005;24(6):634-6. [Medline].

  55. Suzuki G, Itoh Y, Horiuchi Y. Surgical management of eosinophilic fasciitis of the upper extremity. J Hand Surg Br. Jun 1997;22(3):405-7. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

eosinophilic fasciitis, EF, eosinophilia, scleroderma, systemic sclerosis, elevated sedimentation rate, elevated ESR, hypergammaglobulinemia, fascial thickening, hematologic disease, Shulman syndrome, Shulman's syndrome, peripheral eosinophilia, fasciitis, fibrosing disorders, diffuse fasciitis with eosinophilia

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Peter M Henning, DO, MAJ, MC, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Peter M Henning, DO, MAJ, MC is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

George R Mount, MD, MAJ USA MC, Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Attending Physician, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
George R Mount, MD, MAJ USA MC is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Nicholas David Kortan, DO, CPT, MC,, Resident Physician, Department of Internal Medicine, Walter Reed Army Medical Center, Washington, DC
Nicholas David Kortan, DO, CPT, MC, is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Sherif Nasef, MD, Consulting Staff, Department of Internal Medicine, Division of Rheumatology, Lake Havasu Regional Medical Center
Sherif Nasef, MD is a member of the following medical societies: American College of Physicians, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Medical Association
Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Medical Editor

John Varga, MD, Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University
John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.