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Dermatologic Manifestations of Eosinophilic Fasciitis Treatment & Management

  • Author: Brad S Graham, MD; Chief Editor: William D James, MD  more...
 
Updated: Sep 14, 2015
 

Medical Care

Many eosinophilic fasciitis cases respond to corticosteroids (88%, with 25% obtaining complete recovery), although spontaneous resolution is possible. Complete recovery may take up to 1-3 years. No consensus on the treatment of eosinophilic fasciitis exists, but most studies indicate that the best response is with moderate-to-high doses of corticosteroids, especially if started early in the disease course. No set dosing schedule is available, but most studies advocate doses of 0.5-1 mg/kg/d until response, with rapid tapering to alternate day therapy.

Several eosinophilic fasciitis cases exist in the literature of recalcitrant disease to corticosteroids in which adjunctive therapy may be required.

Adjunctive medications for eosinophilic fasciitis include hydroxychloroquine, colchicine, cimetidine, cyclosporin,[17] azathioprine, and methotrexate. A more recent study looked at extracorporal photochemotherapy in the treatment of corticosteroid-resistant cases. After 1 year of therapy, 2 of 3 patients showed considerable improvement when combined with low-dose corticosteroids.[18]

Newer therapies that have been used as corticosteroid adjuncts or as monotherapy for eosinophilic fasciitis include infliximab,[19] cyclophosphamide,[20] dapsone,[21] retinoid-UVA1,[22] and oral psoralen plus UVA (PUVA).[23]

In a promising follow-up report on 3 cases of steroid-resistant eosinophilic fasciitis, all patients showed improvement in skin induration and joint contractures with infliximab at 3 mg/kg every 8 weeks. All patients were in remission in 1-3 years.[24]

A single case report in 2009 reported the use of intravenous immunoglobulin to treat eosinophilic fasciitis.[25]

In a large series of 34 patients with eosinophilic fasciitis, corticosteroids was the standard first-line therapy. In this series, 44% of patients received a second-line therapy adjunctive immunosuppressant, usually methotrexate. The highest chance of complete remission was in patients treated with pulse methylprednisolone at the earliest onset of diagnosis. This also minimized the need for additional immunosuppressive drugs.[26]

A case report in 2102 documented 2 patients with corticosteroid-refractory eosinophilic fasciitis who were successfully treated with D-penicillamine.[27]

A case report in 2015 described a relapse post corticosteroid treatment, which became steroid-resistant and refractory to methotrexate and anti-tumor necrosis factor agent treatment. This patient had a rapid response with sustained remission to the anti-interleukin 6 agent, tocilizumab.[28]

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Surgical Care

Surgical decompression of carpal tunnel syndrome may be required for eosinophilic fasciitis.

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Consultations

A physical therapist may be consulted. Active and passive range of motion therapy of the involved extremities and joints is crucial along with medical therapy to prevent and to treat joint contractures.

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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, Texas Dermatological Society, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Ponciano D Cruz, Jr, MD Professor and Vice-Chair, Paul R Bergstresser Chair, 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: Received consulting fee from RCTS for independent contractor; Received honoraria from Mary Kay Cosmetics for consulting; Received grant/research funds from Galderma for principal investigator.

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

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

  3. Anton E. Failure to demonstrate Borrelia burgdorferi-specific DNA in lesions of eosinophilic fasciitis. Histopathology. 2006 Jul. 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. 2003 Dec. 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. 2004 Aug. 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. 2009 Jun. 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. 2010 May 15. 16(5):5. [Medline].

  8. Silló P, Pintér D, Ostorházi E, Mazán M, Wikonkál N, Pónyai K, et al. Eosinophilic Fasciitis associated with Mycoplasma arginini infection. J Clin Microbiol. 2012 Mar. 50(3):1113-7. [Medline]. [Full Text].

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

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

  11. Dowden AM, Ballas ZK. Eosinophilic fasciitis masquerading as angioedema. Ann Allergy Asthma Immunol. 2009 Mar. 102(3):258-9. [Medline].

  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. 1991 Feb. 18(2):259-63. [Medline].

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

  14. Ganta CC, Chatterjee S, Pohlman B, Hojjati M. Chronic graft-versus-host disease presenting as eosinophilic fasciitis: therapeutic challenges and an additional case. J Clin Rheumatol. 2015 Mar. 21 (2):86-94. [Medline].

  15. Nashel J, Steen V. The use of an elevated aldolase in diagnosing and managing eosinophilic fasciitis. Clin Rheumatol. 2015 Aug. 34 (8):1481-4. [Medline].

  16. 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. 2005 Jan. 184(1):169-74. [Medline].

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

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

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

  20. 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. 2008 Feb. 4(1):81-6. [Medline]. [Full Text].

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

  22. 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].

  23. 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].

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

  25. 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. 2009 Oct. 76(5):572-4. [Medline].

  26. Lebeaux D, Francès C, Barete S, Wechsler B, Dubourg O, Renoux J, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford). 2012 Mar. 51(3):557-61. [Medline].

  27. Manzini CU, Sebastiani M, Giuggioli D, Manfredi A, Colaci M, Cesinaro AM, et al. D-penicillamine in the treatment of eosinophilic fasciitis: case reports and review of the literature. Clin Rheumatol. 2012 Jan. 31(1):183-7. [Medline].

  28. Espinoza F, Jorgensen C, Pers YM. Efficacy of Tocilizumab in the treatment of Eosinophilic fasciitis: Report of one case. Joint Bone Spine. 2015 Jul 7. [Medline].

  29. de Boysson H, Chèze S, Chapon F, Le Mauff B, Auzary C, Geffray L. Eosinophilic fasciitis with paroxysmal nocturnal hemoglobinuria. Joint Bone Spine. 2013 Mar. 80(2):208-10. [Medline].

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

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

  32. 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. 2008 Sep. 27(9):1199-201. [Medline].

  33. Pardal-Fernández JM, Sáez-Méndez L, Rodríguez-Vázquez M, Godes-Medrano B, Iñíguez-De Onzoño L. [Multiple mononeuritis and eosinophilic fasciitis in a patient with idiopathic hypereosinophilic syndrome]. Rev Neurol. 2012 Jan 16. 54(2):100-4. [Medline].

  34. 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. 2007 Sep. 26(9):1445-51. [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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