Journal Mobile Options
Table of Contents
Vol. 213, No. 2, 2006
Issue release date: August 2006
Dermatology 2006;213:93–101

Eosinophilic Fasciitis 30 Years after – What Do We Really Know?

Report of 11 Patients and Review of the Literature

Antic M. · Lautenschlager S. · Itin P.H.
aDepartment of Internal Medicine, General Hospital Waid, and bOutpatient Clinic of Dermatology, General Hospital Triemli, Zurich; cDepartment of Dermatology, University Hospital Basel, Basel, and Kantonsspital Aarau, Aarau, Switzerland

Individual Users: Register with Karger Login Information

Please create your User ID & Password

Contact Information

I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in


Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.


  1. Shulman LE: Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome? J Rheumatol 1974;1:46.
  2. Liou CH, Huang GS, Taylor JA, Juan CJ, Gao HW, Chen CY: Eosinophilic fasciitis in a military recruit: MRI evaluation with clinical correlation. Skeletal Radiol 2003;32:52–57.
  3. Choquet-Kastylevsky G, Kanitakis J, Dumas V, Descotes J, Faure M, Claudy A: Eosinophilic fasciitis and simvastatin. Arch Intern Med 2001;161:1456–1457.
  4. Mosconi S, Streit M, Bronimann M, Braathen LR: Eosinophilic fasciitis (Shulman syndrome). Dermatology 2002;205:204–206.
  5. Granter SR, Barnhill RL, Duray PH: Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol 1996;18:465–473.
  6. Jensen E, Hess B, Hunziker T, Roos F, Helbling A: Eosinophilic fasciitis (Shulman syndrome). Schweiz Med Wochenschr 2000;130:156–160.
  7. Romero AG, Fernandez JG, Calatayud JC: Eosinophilic fasciitis associated with simple traumatism. Acta DermVenereol Croat 2001;9:287–290.
  8. Chazerain P, Vigneron AM, Grossin M, Meyer O, Kahn MF: Posttraumatic diffuse eosinophilic fasciitis accepted for workers’ compensation. Rev Rhum Engl Ed 1997;64:433–434.
  9. Uckun A, Sipahi T, Akgun D, Oksai A: Eosinophilic fasciitis successfully treated with oral hydroxyzine: a new therapeutic use of an old drug? Eur J Pediatr 2002;161:118–119.
  10. Costenbader KH, Kieval RI, Anderson RJ: Eosinophilic fasciitis presenting as pitting edema of the extremities. Am J Med 2001;111:318–320.
  11. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB: Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988;17:221–231.
  12. Romano C, Rubegni P, De Aloe G, Stanghellini E, D’Ascenzo G, Andreassi L, Fimiani M: Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol 2003;17:10–13.
  13. Schiener R, Behrens-Williams SC, Gottlober P, Pillekamp H, Peter RU, Kerscher M: Eosinophilic fasciitis treated with psoralen-ultraviolet A bath photochemotherapy. Br J Dermatol 2000;142:804–807.
  14. Blaser KU, Steiger U, Wursch A, Speck B: Eosinophilic fasciitis with aplastic anemia and Hashimoto’s thyroiditis: review of the literature and report of a typical example. Schweiz Med Wochenschr 1989;119:1899–1906.
  15. French LE, Shapiro M, Junkins-Hopkins JM, Wolfe JT, Rook AH: Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: increased production of interleukin 5 and inhibition by interferon alpha. J Am Acad Dermatol 2003;49:1170–1174.
  16. Naschitz JE, Boss JH, Misselevich I, Yeshurun D, Rosner I: The fasciitis-panniculitis syndromes: clinical and pathologic features. Medicine (Baltimore) 1996;75:6–16.
  17. Kaplinsky N, Bubis JJ, Pras M: Localized eosinophilic fasciitis in a child. J Rheumatol 1980;7:541–543.
  18. Buchanan RR, Gordon DA, Muckle TJ, McKenna F, Kraag G: The eosinophilic fasciitis syndrome after phenytoin (dilantin) therapy. J Rheumatol 1980;7:733–736.
  19. Lattmann J, Adam H, von Hochstetter A, Steurer J, Siegenthaler-Zuber G: Eosinophilic fasciitis (Shulman’s syndrome). Dtsch Med Wochenschr 1990;115:1828–1832.
  20. Granter SR, Barnhill RL, Hewins ME, Duray PH: Identification of Borreliaburgdorferi in diffuse fasciitis with peripheral eosinophilia: borrelial fasciitis. JAMA 1994;272:1283–1285.
  21. Hashimoto Y, Takahashi H, Matsuo S, Hirai K, Takemori N, Nakao M, Miyamoto K, Iizuka H: Polymerase chain reaction of Borrelia burgdorferi flagellin gene in Shulman syndrome. Dermatology 1996;192:136–139.
  22. Bonnotte B, Chauffert B, Caillot D, Martin F, Lorcerie B: Successful treatment with antithymocyte globulin and cyclosporin A of a severe aplastic anaemia associated with an eosinophilic fasciitis. Br J Rheumatol 1998;37:1358–1359.
  23. Michaels RM: Eosinophilic fasciitis complicated by Hodgkin’s disease. J Rheumatol 1982;9:473–476.
  24. Kim H, Kim MO, Ahn MJ, Lee YY, Jung TJ, Choi IY, Kim IS, Park CK: Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma. J Korean Med Sci 2000;15:346–350.
  25. 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;140:1185–1187.
  26. Smiley AM, Husain M, Indenbaum S: Eosinophilic fasciitis in association with thyroid disease: a report of three cases. J Rheumatol 1980;7:871–876.
  27. Varga J, Kahari VM: Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol 1997;9:562–570.
  28. Sherif Nas: Eosinophilic fasciitis.
  29. Huppke P, Wilken B, Brockmann K, Sattler B, Hanefeld F: Eosinophilic fasciitis leading to painless contractures. Eur J Pediatr 2002;161:528–530.
  30. Blauvelt A, Falanga V: Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination. Arch Dermatol 1991;127:1159–1166.
  31. 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;35:299–303.
  32. Moore TL, Zuckner J: Eosinophilic fasciitis. Semin Arthritis Rheum 1980;9:228–235.
  33. 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;151:407–412.
  34. Kahari VM, Heino J, Niskanen L, Fraki J, Uitto J: Eosinophilic fasciitis: increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol 1990;126:613–617.
  35. Mori Y, Kahari VM, Varga J: Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep 2002;4:113–122.
  36. Kim H, Kim MO, Ahn MJ, Lee YY, Jung TJ, Choi IY, Kim IS, Park CK: Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma. J Korean Med Sci 2000;15:346–350.
  37. Enokihara H, Furusawa S, Nakakubo H, Kajitani H, Nagashima S, Saito K, Shishido H, Hitoshi Y, Takatsu K, Noma T, et al: T cells from eosinophilic patients produce interleukin-5 with interleukin-2 stimulation. Blood 1989;73:1809–1813.
  38. Dziadzio L, Kelly EA, Panzer SE, Jarjour N, Huttenlocher A: Cytokine abnormalities in a patient with eosinophilic fasciitis. Ann Allergy Asthma Immunol 2003;90:452–455.
  39. Drosou A, Kirsner RS, Welsh E, Sullivan TP, Kerdel FA: Use of infliximab, an anti-tumor necrosis alpha antibody, for inflammatory dermatoses. J Cutan Med Surg 2003;7:382–386.
  40. Toquet C, Hamidou MA, Renaudin K, Jarry A, Foulc P, Barbarot S, Laboisse C, Mussini JM: In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis. J Rheumatol 2003;30:1811–1815.
  41. Jinnin M, Ihn H, Yazawa N, Asano Y, Yamane K, Tamaki K: Circulating soluble CD40 ligand in patients with eosinophilic fasciitis. Ann Rheum Dis 2003;62:190–191.
  42. Shakoory B, Fitzgerald SM, Lee SA, Chi DS, Krishnaswamy G: The role of human mast cell-derived cytokines in eosinophil biology. J Interferon Cytokine Res 2004;24:271–281.
  43. Moutsopoulos HM, Webber BL, Pavlidis NA, Fostiropoulos G, Goules D, Shulman LE: Diffuse fasciitis with eosinophilia: A clinicopathologic study. Am J Med 1980;68:701–709.
  44. Barnes L, Rodnan GP, Medsger TA, Short D: Eosinophilic fasciitis: a pathologic study of twenty cases. Am J Pathol 1979;96:493–517.
  45. Brent LH, Abruzzo JL: Localized eosinophilic fasciitis in a patient with rheumatoid arthritis. J Rheumatol 1985;12:987–989.
  46. Allen SC: Eosinophilic fasciitis in an African – Possible benefit of chloroquine treatment. Postgrad Med J 1984;60:685–686.
  47. Nawata Y, Sueishi M, Koike T, Tomioka H: Eosinophilic fasciitis with autoimmune features. Arthritis Rheum 1983;26:688.
  48. Helfman T, Falanga V: Eosinophilic fasciitis. Clin Dermatol 1994;12:449–455.
  49. Doyle JA: Eosinophilic fasciitis: extracutaneous manifestations and associations. Cutis 1984;34:259–261.
  50. Kirschstein M, Helmchen U, Jensen R, Kuster RM, Lehmann H: Kidney involvement in a 17-year-old boy with eosinophilic fasciitis. Clin Nephrol 1999;52:183–187.
  51. Naschitz JE, Yeshurun D, Miselevich I, Boss JH: Colitis and pericarditis in a patient with eosinophilic fasciitis: a contribution to the multisystem nature of eosinophilic fasciitis. J Rheumatol 1989;16:688–692.
  52. Mazanec DJ: Eosinophilic fasciitis and pernicious anemia with thyroid antibodies. J Rheumatol 1982;9:742–743.
  53. Imren S, Tuzuner N, Yazici H: Eosinophilic fasciitis with thyroid disease. Clin Exp Rheumatol 1988;6:96–97.
  54. Boiesen M, Keiding LM, Thomsen K: Eosinophilic fasciitis. Report of a case with features of other autoimmune disease. Dermatologica 1983;167:142–144.
  55. Kim SW, Rice L, Champlin R, Udden MM: Aplastic anemia in eosinophilic fasciitis: responses to immunosuppression and marrow transplantation. Haematologia (Budap) 1997;28:131–137.
  56. Chaudhary UB, Eberwine SF, Hege KM: Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: a long relapsing and remitting course. Am J Hematol 2004;75:146–150.
  57. Michaels RM: Eosinophilic fasciitis complicated by Hodgkin’s disease. J Rheumatol 1982;9:473–476.
  58. Junca J, Cuxart A, Tural C, Ojanguren I, Flores A: Eosinophilic fasciitis and non-Hodgkin lymphoma. Eur J Haematol 1994;52:304–306.
  59. Naschitz JE, Yeshurun D, Zuckerman E, Rosenbaum M, Misselevitch I, Shajrawi I, Boss JH: Cancer-associated fasciitis panniculitis. Cancer 1994;73:231–235.
  60. Kowalzick L, Artlett CM, Thoss K, Baum HP, Ziegler H, Mischke D, Blum R, Pönnighaus JM, Quietzsch J: Chronic graft-versus-host-disease-like dermatopathy in a child with CD4+ cell microchimerism. Dermatology 2005;210:68–71.
  61. Bennett RM, Herron A, Keogh L: Eosinophilic fasciitis: case report and review of the literature. Ann Rheum Dis 1977;36:354–359.
  62. Graham BS: Eosinophilic fasciitis.
  63. Solomon G, Barland P, Rifkin H: Eosinophilic fasciitis responsive to cimetidine. Ann Intern Med 1982;97:547–549.
  64. Martinez-Osuna P, Wallach PM, Seleznick MJ, Levin RW, Silveira LH, Jara LJ, Espinoza LR: Treatment of the eosinophilia-myalgia syndrome. Semin Arthritis Rheum 1991;21:110–121.
  65. Guseva NG, Abdykhalykova ZD, Bel’skaia OB, Ivanova MM: Clinical aspects and diagnosis of diffuse eosinophilic fasciitis. Ter Arkh 1986;58:131–135.
  66. Valencia IC, Chang A, Kirsner RS, Kerdel FA: Eosinophilic fasciitis responsive to treatment with pulsed steroids and cyclosporine. Int J Dermatol 1999;38:369–372.

Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 26.50