Clinical Rheumatology

, Volume 26, Issue 9, pp 1445–1451 | Cite as

Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome

  • Yukie Endo
  • Atsushi Tamura
  • Youichiro Matsushima
  • Tomoko Iwasaki
  • Michiko Hasegawa
  • Yayoi Nagai
  • Osamu Ishikawa
Original Article

Abstract

We reported two patients with refractory eosinophilic fasciitis (EF) and provided a systematic review of the literature to determine the clinical variables associated with prognosis of EF. We enrolled 88 cases, whose clinical characteristics were analyzed by separating the patients into two or three groups based on outcome. The incidence of certain clinical and pathological features differed among the groups. In particular, the incidence of morphea-like skin lesions in patients with refractory fibrosis was significantly higher than in patients without refractory fibrosis (p = 0.003). Patients with morphea-like skin lesions were 1.9 times more likely to develop persistent fibrosis than patients without these lesions (95% confidence intervals, 1.5–2.5). A younger age (under 12 years) at onset was associated with a 1.6 times greater risk of residual fibrosis (95% confidence interval, 1.1–2.2). Trunk involvement was associated with a 1.4 times greater risk of residual fibrosis (95% confidence interval, 1.0–2.0). Histopathologically, the presence of dermal fibrosclerosis was associated with a 1.4 times greater risk of refractory fibrosis (95% confidence interval, 1.0–2.1). We consider these clinical characteristics, notably the presence of morphea-like skin lesions may be an important risk factor for developing residual fibrosis in EF patients.

Keywords

Corticosteroid Eosinophilic fasciitis Morphea 

Notes

Acknowledgements

This work was supported by the Ministry of Health Welfare Scleroderma Research Committee, Japan.

References

  1. 1.
    Shulman LE (1975) Diffuse fasciitis with eosinophilia. A new syndrome? Trans Assoc Am Physicians 88:70–86PubMedGoogle Scholar
  2. 2.
    Rodnan GP, DiBartolomeo A, Medsger TA Jr, Barnes EL Jr (1975) Eosinophilic fasciitis: report of 7 cases of a newly recognized scleroderma-like syndrome (abstract). Arthritis Rheum 18:422–423CrossRefGoogle Scholar
  3. 3.
    Doyle JA, Ginsburg WW (1989) Eosinophilic fasciitis. Med Clin North Am 73:1157–1166PubMedGoogle Scholar
  4. 4.
    Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB (1988) Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 17:221–231PubMedCrossRefGoogle Scholar
  5. 5.
    Helfman T, Falanga V (1994) Eosinophilic fasciitis. Clin Dermatol 12:449–455PubMedCrossRefGoogle Scholar
  6. 6.
    Shulman LE (1974) Diffuse fasciitis with eosinophilia. A new syndrome? (abstract). J Rheumatol 1(Suppl 1):46Google Scholar
  7. 7.
    Bennett RM, Herron A, Keogh L (1977) Eosinophilic fasciitis. Case report and review of the literature. Ann Rheum Dis 36:354–359PubMedCrossRefGoogle Scholar
  8. 8.
    Torres VM, George WM (1977) Diffuse eosinophilic fasciitis. A new syndrome or variant of scleroderma? Arch Dermatol 113:1591–1593PubMedCrossRefGoogle Scholar
  9. 9.
    Shewmake SW, Lopez DA, McGlamory JC (1978) The Shulman syndrome. Arch Dermatol 114:556–559PubMedCrossRefGoogle Scholar
  10. 10.
    Weinstein D, Schwartz RA (1978) Eosinophilic fasciitis. Arch Dermatol 114:1047–1049PubMedCrossRefGoogle Scholar
  11. 11.
    Fu TS, Soltani K, Sorensen LB, Levinson D, Lorincz AL (1978) Eosinophilic fasciitis. JAMA 240:451–453PubMedCrossRefGoogle Scholar
  12. 12.
    Fleischmajer R, Jacotot AB, Shore S, Binnick SA (1978) Scleroderma, eosinophilia, and diffuse fasciitis. Arch Dermatol 114:1320–1325PubMedCrossRefGoogle Scholar
  13. 13.
    Chanda JJ, Callen JP, Taylor WB (1978) Diffuse fasciitis with eosinophilia. Arch Dermatol 114:1522–1524PubMedCrossRefGoogle Scholar
  14. 14.
    Tamura T, Saito Y, Ishikawa H (1979) Diffuse fasciitis with eosinophilia: histological and electron microscopic study. Acta Derm Venereol 59:325–331PubMedGoogle Scholar
  15. 15.
    Nassonova VA, Ivanova MM, Akhnazarova VD, Oskilko TG, Bjelle A, Hofer PA, Henriksson KG, Strom T (1979) Eosinophilic fasciitis. Review and report of six cases. Scand J Rheumatol 8:225–233PubMedGoogle Scholar
  16. 16.
    Keczkes K, Goode JD (1979) The Shulman syndrome: report of a further case. Br J Dermatol 100:381–384PubMedCrossRefGoogle Scholar
  17. 17.
    Radvan GH, Coombes BH, Francis PN (1979) Diffuse fasciitis with eosinophilia (Shulman’s disease). Med J Aust 2:397–399PubMedGoogle Scholar
  18. 18.
    Laso FJ, Aparicio M, Diez-Jarilla JL, Gonzalez-Macias J (1980) A new case of eosinophilic fasciitis. Dermatologica 161:211–216PubMedGoogle Scholar
  19. 19.
    Moutsopoulos HM, Webber BL, Pavlidis NA, Fostiropoulos G, Goules D, Shulman LE (1980) Diffuse fasciitis with eosinophilia. A clinicopathologic study. Am J Med 68:701–709PubMedCrossRefGoogle Scholar
  20. 20.
    Barraclough D, Begg MW (1980) Diffuse fasciitis with eosinophilia. Aust NZ J Med 10:333–335Google Scholar
  21. 21.
    Kaplinsky N, Revach M, Katz WA (1980) Eosinophilic fasciitis: report of a case with features of connective tissue diseases. J Rheumatol 7:536–540PubMedGoogle Scholar
  22. 22.
    Smiley AM, Husain M, Indenbaum S (1980) Eosinophilic fasciitis in association with thyroid disease: a report of three cases. J Rheumatol 7:871–876PubMedGoogle Scholar
  23. 23.
    Janin-Mercier A, Bourges M, Fonck-Cussac Y, Bussieres JL, Leblanc B, Delage J (1981) Eosinophilic fasciitis. Ultrastructural study of an early biopsied case. Virchows Arch A Pathol Anat Histopathol 394:177–184CrossRefGoogle Scholar
  24. 24.
    Kent LT, Cramer SF, Moskowitz RW (1981) Eosinophilic fasciitis: clinical, laboratory, and microscopic considerations. Arthritis Rheum 24:677–683PubMedCrossRefGoogle Scholar
  25. 25.
    Hines DA, Friedler EM (1981) Eosinophilic fasciitis: an underdiagnosed syndrome. South Med J 74:643–644PubMedGoogle Scholar
  26. 26.
    Thornell LE, Bjelle A (1981) Eosinophilic fasciitis: an ultrastructural and immunohistochemical study of the intermediate filament protein skeletin in regenerating muscle fibres. Neuropathol Appl Neurobiol 7:435–449PubMedGoogle Scholar
  27. 27.
    Lewkonia RM, Marx LH, Atkinson MH (1982) Granulomatosis vasculitis in the syndrome of diffuse fasciitis with eosinophilia. Arch Intern Med 142:73–75PubMedCrossRefGoogle Scholar
  28. 28.
    Aelion JA, Wysenbeek AJ, Avidor I, Blum I (1982) Eosinophilic fasciitis (Shulman syndrome) with prominent synovitis. Isr J Med Sci 18:793–797PubMedGoogle Scholar
  29. 29.
    Caspi D, Fishel R, Varon M, Yona E, Baratz M, Yaron M (1982) Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil 21:218–221PubMedCrossRefGoogle Scholar
  30. 30.
    Sills EM (1982) Diffuse fasciitis with eosinophilia in childhood. Johns Hopkins Med J 151:203–207PubMedGoogle Scholar
  31. 31.
    Boiesen M, Keiding LM, Thomsen K (1983) Eosinophilic fasciitis. Report of a case with features of other autoimmune disease. Dermatologica 167:142–144PubMedGoogle Scholar
  32. 32.
    Naguwa SM, Robbins DL, Castles JJ (1983) Eosinophilic fasciitis: a distinct clinical entity? Am J Med Sci 286:32–35PubMedCrossRefGoogle Scholar
  33. 33.
    Patrone NA, Kredich DW (1984) Eosinophilic fasciitis in a child. Am J Dis Child 138:363–365PubMedGoogle Scholar
  34. 34.
    De Jonge-Bok JM, Steven MM, Eulderink F, Cats A (1984) Diffuse (eosinophilic) fasciitis. A series of six cases. Clin Rheumatol 3:365–373PubMedCrossRefGoogle Scholar
  35. 35.
    Allen SC (1984) Eosinophilic fasciitis in an African-possible benefit of chloroquine treatment. Postgrad Med J 60:685–686PubMedGoogle Scholar
  36. 36.
    Jacobs MB (1985) Eosinophilic fasciitis, reactive hepatitis, and splenomegaly. Arch Intern Med 145:162–163PubMedCrossRefGoogle Scholar
  37. 37.
    Frayha RA, Atiyah F, Karam P, Ali Ahmed Z, Salman SM (1985) Eosinophilic fasciitis terminating as progressive systemic sclerosis in a child. Dermatologica 171:291–294PubMedGoogle Scholar
  38. 38.
    Sartor O, Sander GE (1985) Unusual variant of eosinophilic fasciitis. South Med J 78:1387–1389PubMedGoogle Scholar
  39. 39.
    Jones HR Jr, Beetham WP Jr, Silverman ML, Margles SW (1986) Eosinophilic fasciitis and the carpal tunnel syndrome. J Neurol Neurosurg Psychiatry 49:324–327PubMedGoogle Scholar
  40. 40.
    Williams HJ, Ziter FA, Banta CA (1986) Childhood eosinophilic fasciitis—progression to linear scleroderma. J Rheumatol 13:961–962PubMedGoogle Scholar
  41. 41.
    Thomson GT, MacDougall B, Watson PH, Chalmers IM (1989) Eosinophilic fasciitis in a pair of siblings. Arthritis Rheum 32:96–99PubMedCrossRefGoogle Scholar
  42. 42.
    Pressly TA, Treadwell EL, Ansbacher LE, Winkler A, Hoyt TD, Walker SE (1989) Eosinophilic fasciitis with porphyria cutanea tarda and progressive destructive arthritis. J Rheumatol 16:390–393PubMedGoogle Scholar
  43. 43.
    Amdur HS, Levin RE (1989) Eosinophilic fasciitis during pregnancy. Obstet Gynecol 73:843–847PubMedGoogle Scholar
  44. 44.
    Falanga V, Soter NA, Kerdel FA (1989) Increased plasma histamine level in eosinophilic fasciitis. Arch Dermatol 125:805–808PubMedCrossRefGoogle Scholar
  45. 45.
    Markusse HM, Breedveld FC (1989) Rheumatoid arthritis with eosinophilic fasciitis and pure red cell aplasia. J Rheumatol 16:1383–1384PubMedGoogle Scholar
  46. 46.
    Markusse HM, Dijkmans BA, Fibbe WE (1990) Eosinophilic fasciitis after allogeneic bone marrow transplantation. J Rheumatol 17:692–694PubMedGoogle Scholar
  47. 47.
    Chan LS, Hanson CA, Cooper KD (1991) Concurrent eosinophilic fasciitis and cutaneous T-cell lymphoma. Eosinophilic fasciitis as a paraneoplastic syndrome of T-cell malignant neoplasms? Arch Dermatol 127:862–865PubMedCrossRefGoogle Scholar
  48. 48.
    Loeliger AE, van den Brink HR, Derksen RH, Bijlsma JW (1991) Eosinophilic fasciitis presenting with a reactive hepatitis. Clin Rheumatol 10:440–444PubMedCrossRefGoogle Scholar
  49. 49.
    Ching DW, Leibowitz MR (1992) Ketotifen—a therapeutic agent of eosinophilic fasciitis? J Intern Med 231:555–559PubMedCrossRefGoogle Scholar
  50. 50.
    Farrington ML, Haas JE, Nazar-Stewart V, Mellins ED (1993) Eosinophilic fasciitis in children frequently progresses to scleroderma-like cutaneous fibrosis. J Rheumatol 20:128–132PubMedGoogle Scholar
  51. 51.
    Wong AL, Anderson-Wilms N, Mortensen SE, Colburn KK (1993) Eosinophilic fasciitis in association with chronic vasculitic-like leg ulcerations. Clin Rheumatol 12:85–88PubMedCrossRefGoogle Scholar
  52. 52.
    Castanet J, Lacour JP, Perrin C, Taillan B, Dubois D, Ortonne JP (1994) Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody. Dermatology 189:304–307PubMedCrossRefGoogle Scholar
  53. 53.
    Watts RA, Merry P (1994) Familial eosinophilic fasciitis and breast cancer. Br J Rheumatol 33:93–94PubMedCrossRefGoogle Scholar
  54. 54.
    O’Laughlin TJ, Klima RR, Kenney DE (1994) Rehabilitation of eosinophilic fasciitis. A case report. Am J Phys Med Rehabil 73:286–292PubMedCrossRefGoogle Scholar
  55. 55.
    al-Shaikh A, Freeman C, Avruch L, McKendry RJ (1994) Use of magnetic resonance imaging in diagnosing eosinophilic fasciitis. Report of two cases. Arthritis Rheum 37:1602–1608PubMedCrossRefGoogle Scholar
  56. 56.
    Takeda S, Takazakura E, Fukui Y (1995) Tubulointerstitial nephritis in a patient with eosinophilic fasciitis and IgA nephropathy. Nephron 69:314–317PubMedGoogle Scholar
  57. 57.
    Fujimoto M, Sato S, Ihn H, Kikuchi K, Yamada N, Takehara K (1995) Serum aldolase level is a useful indicator of disease activity in eosinophilic fasciitis. J Rheumatol 22:563–565PubMedGoogle Scholar
  58. 58.
    Ferlazzo B, Quattrocchi P, Carretti C, La Rosa G, Barresi G (1995) Eosinophilic fasciitis: report of a case diagnosed 14 years after its onset. Minerva Med 86:495–498PubMedGoogle Scholar
  59. 59.
    Stork J, Nemcova D, Hoza J, Kodetova D (1996) Eosinophilic fasciitis in an adolescent girl with lymphadenopathy and vitiligo-like and linear scleroderma-like changes. A case report. Clin Exp Rheumatol 14:337–341PubMedGoogle Scholar
  60. 60.
    Nakajima H, Fujiwara S, Shinoda K, Ohsawa N (1997) Magnetic resonance imaging and serum aldolase concentration in eosinophilic fasciitis. Intern Med 36:654–656PubMedGoogle Scholar
  61. 61.
    Moriguchi M, Terai C, Kuroki S, Tanaka E, Someya N, Tsunoda Y, Kashiwazaki S (1998) Eosinophilic fasciitis complicated with peripheral polyneuropathy. Intern Med 37:417–420PubMedGoogle Scholar
  62. 62.
    Naschitz JE, Misselevich I, Rosner I, Yeshurun D, Weiner P, Amar M, Amato L, Ciompi ML, Boss JH (1999) Lymph-node-based malignant lymphoma and reactive lymphadenopathy in eosinophilic fasciitis. Am J Med Sci 318:343–349PubMedCrossRefGoogle Scholar
  63. 63.
    Killen JW, Swift GL, White RJ (2000) Eosinophilic fasciitis with pulmonary and pleural involvement. Postgrad Med J 76:36–37PubMedCrossRefGoogle Scholar
  64. 64.
    Kim H, Kim MO, Ahn MJ, Lee YY, Jung TJ, Choi IY, Kim IS, Park CK (2000) Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma. J Korean Med Sci 15:346–350PubMedGoogle Scholar
  65. 65.
    Huemer M, Seeber A, Huemer C (2000) Scleroderma-like syndrome in a child: eosinophilic fasciitis or scleredema adultorum? Eur J Pediatr 159:520–522PubMedCrossRefGoogle Scholar
  66. 66.
    Vannucci P, Gaeta P, Riccioni N (2001) Association of eosinophilic fasciitis and epileptic seizure. Clin Rheumatol 20:223–224PubMedCrossRefGoogle Scholar
  67. 67.
    Mosconi S, Streit M, Bronimann M, Braathen LR (2002) Eosinophilic fasciitis (Shulman syndrome). Dermatology 205:204–206PubMedCrossRefGoogle Scholar
  68. 68.
    Liou CH, Huang GS, Taylor JA, Juan CJ, Gao HW, Chen CY (2003) Eosinophilic fasciitis in a military recruit: MRI evaluation with clinical correlation. Skelet Radiol 32:52–57CrossRefGoogle Scholar
  69. 69.
    Chaudhary UB, Eberwine SF, Hege KM (2004) Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: a long relapsing and remitting course. Am J Hematol 75:146–150PubMedCrossRefGoogle Scholar
  70. 70.
    Chan V, Soans B, Mathers D (2004) Ultrasound and magnetic resonance imaging features in a patient with eosinophilic fasciitis. Australas Radiol 48:414–417PubMedCrossRefGoogle Scholar
  71. 71.
    Ustun C, Ho G Jr (2004) Eosinophilic fasciitis after allogeneic stem cell transplantation: a case report and review of the literature. Leuk Lymphoma 45:1707–1709PubMedCrossRefGoogle Scholar
  72. 72.
    Bukiej A, Dropinski J, Dyduch G, Szczeklik A (2005) Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol 24:634–636PubMedCrossRefGoogle Scholar
  73. 73.
    Carneiro S, Brotas A, Lamy F, Lisboa F, Lago E, Azulay D, Cuzzi T, Ramos-e-Silva M (2005) Eosinophilic fasciitis (Shulman syndrome). Cutis 75:228–232PubMedGoogle Scholar
  74. 74.
    Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL (2005) Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum. J Am Acad Dermatol 53:591–601PubMedCrossRefGoogle Scholar
  75. 75.
    Wu L, Xia YP, Roth SI, Gruskin E, Mustoe TA (1999) Transforming growth factor-beta1 fails to stimulate wound healing and impairs its signal transduction in an aged ischemic ulcer model: importance of oxygen and age. Am J Pathol 154:301–309PubMedGoogle Scholar
  76. 76.
    Kahari L, Jimenez SA (1996) Increased expression of transforming growth factor-beta1, fibronectin, and Types I, III, and VI collagen genes in fascial fibroblasts from patients with diffuse fasciitis with eosinophilia. J Rheumatol 23:482–486PubMedGoogle Scholar
  77. 77.
    Barnes L, Rodnan GP, Medsger TA, Short D (1979) Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol 96:493–517PubMedGoogle Scholar
  78. 78.
    Falanga V, Medsger TA Jr (1987) Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis. J Am Acad Dermatol 17:648–656PubMedCrossRefGoogle Scholar
  79. 79.
    Naschitz JE, Yeshurun D, Zuckerman E, Rosenbaum M, Misselevitch I, Shajrawi I, Boss JH (1994) Cancer-associated fasciitis panniculitis. Cancer 73:231–235PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2007

Authors and Affiliations

  • Yukie Endo
    • 1
  • Atsushi Tamura
    • 1
  • Youichiro Matsushima
    • 1
  • Tomoko Iwasaki
    • 1
  • Michiko Hasegawa
    • 1
  • Yayoi Nagai
    • 1
  • Osamu Ishikawa
    • 1
  1. 1.Department of DermatologyGunma University Graduate School of MedicineMaebashiJapan

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