UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

Eosinophilic fasciitis

Authors
Simon M Helfgott, MD
John Varga, MD
Section Editor
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Eosinophilic fasciitis (EF, also called Shulman syndrome) is an uncommon disorder of unknown etiology and poorly understood pathogenesis [1]. EF is characterized in its early phase by limb or trunk erythema and edema and later by collagenous thickening of the subcutaneous fascia. Eosinophilia is a prominent laboratory finding in the early phase, although not always present in active early cases and less prominent in later stages [2].

This topic will review the clinical manifestations, pathology, diagnosis, differential diagnosis, and treatment of EF. The approach to the patient with eosinophilia, and other major causes of eosinophilia are discussed separately. (See "Approach to the patient with unexplained eosinophilia" and "Eosinophil biology and causes of eosinophilia".)

ETIOLOGY

The etiology of eosinophilic fasciitis (EF) is unknown. The following have been suggested as possible triggers or factors associated with EF [3]:

Strenuous exercise [1]

Initiation of hemodialysis [4]

              

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Feb 2017. | This topic last updated: Fri Jan 13 00:00:00 GMT+00:00 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 1975; 88:70.
  2. 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; 17:648.
  3. Pinal-Fernandez I, Selva-O' Callaghan A, Grau JM. Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev 2014; 13:379.
  4. Florell SR, Egan CA, Gregory MC, et al. Eosinophilic fasciitis occurring four weeks after the onset of dialysis in a renal failure patient. J Cutan Med Surg 2001; 5:33.
  5. Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol 1996; 18:465.
  6. Hashimoto Y, Takahashi H, Matsuo S, et al. Polymerase chain reaction of Borrelia burgdorferi flagellin gene in Shulman syndrome. Dermatology 1996; 192:136.
  7. Mosconi S, Streit M, Brönimann M, Braathen L. Eosinophilic fasciitis (Shulman syndrome). Dermatology 2002; 205:204.
  8. Long H, Zhang G, Wang L, Lu Q. Eosinophilic Skin Diseases: A Comprehensive Review. Clin Rev Allergy Immunol 2016; 50:189.
  9. Bachmeyer C, Monge M, Dhôte R, et al. Eosinophilic fasciitis following idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Hashimoto's disease. Dermatology 1999; 199:282.
  10. Farrell AM, Ross JS, Bunker CB. Eosinophilic fasciitis associated with autoimmune thyroid disease and myelodysplasia treated with pulsed methylprednisolone and antihistamines. Br J Dermatol 1999; 140:1185.
  11. Imren S, Tüzüner N, Yazici H. Eosinophilic fasciitis with thyroid disease. Clin Exp Rheumatol 1988; 6:96.
  12. Boiesen M, Keiding LM, Thomsen K. Eosinophilic fasciitis. Report of a case with features of other autoimmune disease. Dermatologica 1983; 167:142.
  13. Smiley AM, Husain M, Indenbaum S. Eosinophilic fasciitis in association with thyroid disease: a report of three cases. J Rheumatol 1980; 7:871.
  14. Rodnan GP, DiBartolomeo A, Medsger TA Jr. Proceedings: Eosinophilic fasciitis. Report of six cases of a newly recognized scleroderma-like syndrome. Arthritis Rheum 1975; 18:525.
  15. Doyle JA, Ginsburg WW. Eosinophilic fasciitis. Med Clin North Am 1989; 73:1157.
  16. 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; 18:259.
  17. Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988; 17:221.
  18. Huppke P, Wilken B, Brockmann K, et al. Eosinophilic fasciitis leading to painless contractures. Eur J Pediatr 2002; 161:528.
  19. Nassonova VA, Ivanova MM, Akhnazarova VD, et al. Eosinophilic fasciitis. Review and report of six cases. Scand J Rheumatol 1979; 8:225.
  20. Jones HR Jr, Beetham WP Jr, Silverman ML, Margles SW. Eosinophilic fasciitis and the carpal tunnel syndrome. J Neurol Neurosurg Psychiatry 1986; 49:324.
  21. Moriguchi M, Terai C, Kuroki S, et al. Eosinophilic fasciitis complicated with peripheral polyneuropathy. Intern Med 1998; 37:417.
  22. Killen JW, Swift GL, White RJ. Eosinophilic fasciitis with pulmonary and pleural involvement. Postgrad Med J 2000; 76:36.
  23. Rizzo S. Eosinophilic pleuropericarditis and fasciitis. A new case. Monaldi Arch Chest Dis 2002; 57:311.
  24. Kirschstein M, Helmchen U, Jensen R, et al. Kidney involvement in a 17-year-old boy with eosinophilic fasciitis. Clin Nephrol 1999; 52:183.
  25. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol 2012; 26:449.
  26. Doyle JA, Connolly SM, Hoagland HC. Hematologic disease in scleroderma syndromes. Acta Derm Venereol 1985; 65:521.
  27. Hoffman R, Dainiak N, Sibrack L, et al. Antibody-mediated aplastic anemia and diffuse fasciitis. N Engl J Med 1979; 300:718.
  28. Littlejohn GO, Keystone EC. Eosinophilic fasciitis and aplastic anaemia. J Rheumatol 1980; 7:730.
  29. Chaudhary UB, Eberwine SF, Hege KM. Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: a long relapsing and remitting course. Am J Hematol 2004; 75:146.
  30. Khanna D, Verity A, Grossman JM. Eosinophilic fasciitis with multiple myeloma: a new haematological association. Ann Rheum Dis 2002; 61:1111.
  31. Jacob SE, Lodha R, Cohen JJ, et al. Paraneoplastic eosinophilic fasciitis: a case report. Rheumatol Int 2003; 23:262.
  32. Haddad H, Sundaram S, Magro C, Gergis U. Eosinophilic fasciitis as a paraneoplastic syndrome, a case report and review of the literature. Hematol Oncol Stem Cell Ther 2014; 7:90.
  33. Ferguson JS, Bosworth J, Min T, et al. Eosinophilic fasciitis associated with hypereosinophilia, abnormal bone-marrow karyotype and inversion of chromosome 5. Clin Exp Dermatol 2014; 39:150.
  34. de Masson A, Bouaziz JD, Peffault de Latour R, et al. Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature. Medicine (Baltimore) 2013; 92:69.
  35. Naschitz JE, Misselevich I, Rosner I, et al. Lymph-node-based malignant lymphoma and reactive lymphadenopathy in eosinophilic fasciitis. Am J Med Sci 1999; 318:343.
  36. Eklund KK, Anttila P, Leirisalo-Repo M. Eosinophilic fasciitis, myositis and arthritis as early manifestations of peripheral T-cell lymphoma. Scand J Rheumatol 2003; 32:376.
  37. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol 1979; 96:493.
  38. Fredens K, Dybdahl H, Dahl R, Baandrup U. Extracellular deposit of the cationic proteins ECP and EPX in tissue infiltrations of eosinophils related to tissue damage. APMIS 1988; 96:711.
  39. Falanga V, Soter NA, Kerdel FA. Increased plasma histamine level in eosinophilic fasciitis. Arch Dermatol 1989; 125:805.
  40. Huang KW, Chen XH. Pathology of eosinophilic fasciitis and its relation to polymyositis. Can J Neurol Sci 1987; 14:632.
  41. Moulton SJ, Kransdorf MJ, Ginsburg WW, et al. Eosinophilic fasciitis: spectrum of MRI findings. AJR Am J Roentgenol 2005; 184:975.
  42. De Clerck LS, Degryse HR, Wouters E, et al. Magnetic resonance imaging in the evaluation of patients with eosinophilic fasciitis. J Rheumatol 1989; 16:1270.
  43. Ronneberger M, Janka R, Schett G, Manger B. Can MRI substitute for biopsy in eosinophilic fasciitis? Ann Rheum Dis 2009; 68:1651.
  44. Nakajima H, Fujiwara S, Shinoda K, Ohsawa N. Magnetic resonance imaging and serum aldolase concentration in eosinophilic fasciitis. Intern Med 1997; 36:654.
  45. al-Shaikh A, Freeman C, Avruch L, McKendry RJ. Use of magnetic resonance imaging in diagnosing eosinophilic fasciitis. Report of two cases. Arthritis Rheum 1994; 37:1602.
  46. Baumann F, Brühlmann P, Andreisek G, et al. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol 2005; 184:169.
  47. Kim HJ, Lee SW, Kim GJ, Lee JH. Usefulness of FDG PET/CT in the diagnosis of eosinophilic fasciitis. Clin Nucl Med 2014; 39:801.
  48. Kissin EY, Garg A, Grayson PC, et al. Ultrasound assessment of subcutaneous compressibility: a potential adjunctive diagnostic tool in eosinophilic fasciitis. J Clin Rheumatol 2013; 19:382.
  49. Herson S, Brechignac S, Piette JC, et al. Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma. Am J Med 1990; 88:598.
  50. Mori Y, Kahari VM, Varga J. Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep 2002; 4:113.
  51. Allen JA, Peterson A, Sufit R, et al. Post-epidemic eosinophilia-myalgia syndrome associated with L-tryptophan. Arthritis Rheum 2011; 63:3633.
  52. Alonso-Ruiz A, Zea-Mendoza AC, Salazar-Vallinas JM, et al. Toxic oil syndrome: a syndrome with features overlapping those of various forms of scleroderma. Semin Arthritis Rheum 1986; 15:200.
  53. Kaufman LD, Krupp LB. Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia. Curr Opin Rheumatol 1995; 7:560.
  54. Posada de la Paz M, Philen RM, Borda AI. Toxic oil syndrome: the perspective after 20 years. Epidemiol Rev 2001; 23:231.
  55. Schaffer JV, McNiff JM, Seropian S, et al. Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum. J Am Acad Dermatol 2005; 53:591.
  56. Lebeaux D, Francès C, Barete S, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford) 2012; 51:557.
  57. 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; 47:29.
  58. Endo Y, Tamura A, Matsushima Y, et al. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol 2007; 26:1445.
  59. Schiener R, Behrens-Williams SC, Gottlöber P, et al. Eosinophilic fasciitis treated with psoralen-ultraviolet A bath photochemotherapy. Br J Dermatol 2000; 142:804.
  60. Pimenta S, Bernardes M, Bernardo A, et al. Intravenous immune globulins to treat eosinophilic fasciitis: a case report. Joint Bone Spine 2009; 76:572.
  61. Bonnotte B, Chauffert B, Caillot D, et al. Successful treatment with antithymocyte globulin and cyclosporin A of a severe aplastic anaemia associated with an eosinophilic fasciitis. Br J Rheumatol 1998; 37:1358.
  62. Caspi D, Fishel R, Varon M, et al. Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil 1982; 21:218.
  63. Jones AC, Doherty M. Eosinophilic fasciitis with late onset arthritis responsive to sulfasalazine. J Rheumatol 1993; 20:750.
  64. 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; 49:1184.
  65. Scheinberg M, Hamerschlak N, Kutner JM, et al. Rituximab in refractory autoimmune diseases: Brazilian experience with 29 patients (2002-2004). Clin Exp Rheumatol 2006; 24:65.
  66. Suzuki G, Itoh Y, Horiuchi Y. Surgical management of eosinophilic fasciitis of the upper extremity. J Hand Surg Br 1997; 22:405.
  67. Neumeister MW, Robertson GA. Therapeutic fasciectomy for eosinophilic fasciitis. Ann Plast Surg 1998; 41:208.