Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Eosinophilic Fasciitis Workup

  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 12, 2016
 

Laboratory Studies

Characteristic laboratory findings of eosinophilic fasciitis (EF) include the following:

  • Peripheral blood eosinophilia is present in 61%-83% of patients. The degree of eosinophilia is variable over time, even in the absence of specific therapy.[18, 39, 40]
  • Hypergammaglobulinemia is characteristic, although this finding varies widely by case series, occurring in 18%-67% of patients. It is most often due to a polyclonal increase in immunoglobulin G.[19, 20, 39]
  • An increase in the erythrocyte sedimentation rate (ESR) is found in 29%-70% of cases.[19]

Additional laboratory findings of eosinophilic fasciitis include the following:[18, 20, 39]

  • Serum creatine kinase and aldolase levels are generally normal.
  • Rheumatoid factor (RF) and antinuclear antibodies are occasionally positive.
  • Hematologic abnormalities and disease are associated with eosinophilic fasciitis. Aplastic anemia, although rare, is the most frequent common associated hematological complication, but cases have been described with thrombocytopenia, hemolytic anemia, pernicious anemia, lymphoma, and leukemia.[41, 42]
  • Borrelia serology or polymerase chain reaction (PCR) findings are occasionally positive and may suggest a treatable etiology. However, as discussed above, the exact correlation between eosinophilic fasciitis and Borrelia remains unclear.[26, 27]
  • Metalloproteinase 1 (TIMP-1) may be a new serological marker of disease activity.[12]
Next

Imaging Studies

MRI is the imaging modality of choice. MRI of the involved areas shows characteristic findings of fascial thickening, abnormal signal intensity, and contrast enhancement. Additionally, MRI aids in making the diagnosis, locating the biopsy site, and monitoring the response to treatment.[43, 44, 45, 46]

Although it has not been used frequently or studied extensively in eosinophilic fasciitis, one case report has shown that ultrasonography can aid in early diagnosis.[47] According to a study by Kissin et al that included 12 patients with eosinophilic fasciitis, a 12-MHz, B-mode ultrasound may be used to measure subcutaneous compressibility and thereby serve as an adjunctive tool to distinguish eosinophilic fasciitis from diffuse systemic sclerosis, especially when tissue sampling is less feasible or when the result of tissue sampling is equivocal.[48]

Previous
Next

Other Tests

While eosinophilic fasciitis is generally not associated with myositis or myopathy, electromyography has occasionally been performed, and findings may be abnormal in the presence of normal serum muscle enzymes.[20]

Pulmonary function testing may show a restrictive pattern in patients with severe truncal involvement.[20]

Previous
Next

Procedures

Definitive diagnosis relies on a full-thickness incisional skin biopsy. The specimen should include the skin, fat, fascia, and superficial muscle in continuity. Biopsy is especially important in an atypical presentation.[49, 50, 51]

Previous
Next

Histologic Findings

Inflammation, edema, thickening, and sclerosis of the fascia are hallmarks of eosinophilic fasciitis. Acute findings include infiltration of deep fascia and an adjacent subcutis layer with lymphocytes, plasma cells, histiocytes, and eosinophils. Distribution of the eosinophils in the fascia may be focal, and a close relationship appears to exist between blood and tissue eosinophilia. In the deeper portions of the panniculus, a similar infiltrate is found in the fibrous septa and at the periphery of the fat lobules. Deep in the fascia, the inflammatory infiltrate can extend into the epimysium, perimysium, and endomysium. In addition, vascular cuffing with lymphocytes and plasma cells is often seen.[10, 14, 52]

As the disease progresses, inflammatory changes are replaced by generalized sclerosis and thickening of the fascia and adjacent tissue layers. The sclerosis can be dense with hyalinized collagen bands running parallel to the fascia and small foci of fat cells trapped between them.[39, 53] See the image below.

Eosinophilic fasciitis. Top: In this gross specimeEosinophilic fasciitis. Top: In this gross specimen, the dermis (A), subcutaneous adipose tissue (B), and skeletal muscle do not appear unusual. However, the fascia (D) is markedly thickened. Bottom left: The gross findings are recapitulated in this low-power photomicrograph. The epidermis, dermis (A), and subcutaneous adipose tissue are not remarkable in this case. The fascia (D) is markedly thickened and focally infiltrated by inflammatory cells (E). The small amount of skeletal muscle (C) appears normal (hematoxylin and eosin stain at low power). Bottom right: A close-up photograph of a portion of the fascia showing mostly edematous cellular connective tissue (F). It is focally infiltrated by inflammatory cells, including lymphocytes, plasma cells, and histiocytes. The more intensely stained hypocellular pink bands across the top of the field (G) are part of an interstitial exudate of fibrin (hematoxylin and eosin stain at medium power).
Previous
 
 
Contributor Information and Disclosures
Author

Peter M Henning, DO MAJ, US Marine Corps; Fellow, Department of Rheumatology, Walter Reed Army Medical Center

Peter M Henning, DO is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

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, American Medical Association

Disclosure: Nothing to disclose.

George R Mount, MD MAJ USA MC, Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Attending Physician, Department of Rheumatology, Madigan Army Medical Center, Tacoma, WA

George R Mount, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Nicholas David Kortan, DO Fellow in Rheumatology, Walter Reed National Military Medical Center

Nicholas David Kortan, DO is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of 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 for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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 and Translational Research, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The opinions or assertions contained here are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

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

  2. 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). 2011 Nov 25. [Medline].

  3. Chun JH, Lee KH, Sung MS, Park CJ. Two cases of eosinophilic fasciitis. Ann Dermatol. 2011 Feb. 23(1):81-4. [Medline]. [Full Text].

  4. 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].

  5. Wright NA, Mazori DR, Patel M, Merola JF, Femia AN, Vleugels RA. Epidemiology and Treatment of Eosinophilic Fasciitis: An Analysis of 63 Patients From 3 Tertiary Care Centers. JAMA Dermatol. 2016 Jan 1. 152 (1):97-9. [Medline].

  6. 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. 2001 Jan. 28(1):75-80. [Medline].

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

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

  9. 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. 1990 May. 126(5):613-7. [Medline].

  10. 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. 1990 Mar. 94(3):365-71. [Medline].

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

  12. 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].

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

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

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

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

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

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

  19. 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].

  20. 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. 2008 Jan. 47(1):29-35. [Medline].

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

  41. 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].

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

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

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

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

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

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

  48. Kissin EY, Garg A, Grayson PC, Dubreuil M, Vradii D, York M, et al. Ultrasound assessment of subcutaneous compressibility: a potential adjunctive diagnostic tool in eosinophilic fasciitis. J Clin Rheumatol. 2013 Oct. 19(7):382-5. [Medline].

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

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

  51. 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].

  52. 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. 1982 Feb. 106(2):85-91. [Medline].

  53. 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. 1990 May. 126(5):613-7. [Medline].

  54. Manzini C, Sebastiani M, Giuggioli D, Manfredi A, Colaci M, Cesinaro A, et al. D-penicillamine in the treatment of eosinophilic fasciitis: case reports and review of the literature. Clin Rheumatol. 2011 Oct 12. [Medline].

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

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

  57. 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].

  58. 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].

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

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

  61. 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].

  62. de Masson A, Bouaziz JD, Peffault de Latour R, Benhamou Y, Moluçon-Chabrot C, Bay JO, et al. Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature. Medicine (Baltimore). 2013 Mar. 92(2):69-81. [Medline].

Previous
Next
 
The arm of this patient demonstrates the puckered, so-called orange-peel or cobblestone skin that may occur in eosinophilic fasciitis.
The skin of the patient's back appears shiny due to the stretched dermis overlying an inflamed fascia. Mild diffuse hyperpigmentation is present, along with a U-shaped area of hypopigmentation extending approximately from T10 to L4.
The skin of the abdomen and breasts is shiny and taut. The thigh reveals puckering or cobblestoning of the overlying dermis due to scattered retraction from scarred fascia.
Eosinophilic fasciitis. Top: In this gross specimen, the dermis (A), subcutaneous adipose tissue (B), and skeletal muscle do not appear unusual. However, the fascia (D) is markedly thickened. Bottom left: The gross findings are recapitulated in this low-power photomicrograph. The epidermis, dermis (A), and subcutaneous adipose tissue are not remarkable in this case. The fascia (D) is markedly thickened and focally infiltrated by inflammatory cells (E). The small amount of skeletal muscle (C) appears normal (hematoxylin and eosin stain at low power). Bottom right: A close-up photograph of a portion of the fascia showing mostly edematous cellular connective tissue (F). It is focally infiltrated by inflammatory cells, including lymphocytes, plasma cells, and histiocytes. The more intensely stained hypocellular pink bands across the top of the field (G) are part of an interstitial exudate of fibrin (hematoxylin and eosin stain at medium power).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.