HSS Journal

, Volume 4, Issue 1, pp 81–86 | Cite as

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
  • Virginia Florina Haiduc
  • Doruk ErkanEmail author
  • Kyriakos Kirou
  • Sherri Birchansky
  • Josephine Park
  • Moris J. Danon

Case presentation

A 65-year-old white man who carried the diagnosis of eosinophilic fasciitis (EF) presented with worsening neck, shoulder, and back stiffness over the last 3 years. His symptoms started 8 years ago with abdominal muscle tightness and skin thickness, which progressed to upper and lower extremities within weeks. During this period, he also complained of exertional muscle pain, especially after prolonged exercise. Based on peripheral eosinophilia and a muscle biopsy, he was diagnosed with EF and treated successfully with high-dose corticosteroids (CS). However, each attempt to decrease CS dose below 20 mg resulted in worsening of his muscle tightness, especially around proximal upper extremities and neck muscles. Five years before the presentation, he used hydroxychloroquine for 8 months and methotrexate for another 8 months as steroid tapering agents, with no benefit. Three years before the presentation, he developed CS myopathy and thus CS was stopped. Six months before...

Key words

eosinophilic fasciitis cyclophosphamide necrotizing vasculitis 



Antinuclear antibodies


Cytoplasmic antineutrophil cytoplasmic antibody


C-reactive protein




Computed tomography




Eosinophilic fasciitis


Eosinophilia-myalgia syndrome


Idiopathic hypereosinophilic syndrome


Human immunodeficiency virus

IgM, IgG, IgA

Immunoglobulin M, G, A






Intravenous immunoglobulins


Membrane attack complex


Myeloperoxidase antibody


Magnetic resonance angiography


Magnetic resonance imaging


Perinuclear antineutrophil cytoplasmic antibody


Platelet-derived growth factor receptor alpha


Proteinase 3 antibodies


Psoralen plus ultraviolet A


Range of motion


Selective Immunoglobulin M Deficiency


Transforming growth factor-beta


  1. 1.
    Shulman LE (1974) Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome? J Rheumatol(Suppl 1):82Google Scholar
  2. 2.
    Shulman LE (1970) Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 88:70–86Google Scholar
  3. 3.
    Choquet-Kastylevsky G, Kanitakis J, Dumas V, et al. (2001) Eosinophilic fasciitis and simvastatin. Arch Intern Med 161:1456–1457PubMedCrossRefGoogle Scholar
  4. 4.
    DeGiovanni C, Chard M, Woollons A (2006) Eosinophilic fasciitis secondary to treatment with atorvastatin. Clin Exp Dermatol 31:131–132PubMedCrossRefGoogle Scholar
  5. 5.
    Buchanan RR, Gordon DA, Muckle TJ, et al. (1980) The eosinophilic fasciitis syndrome after phenytoin (dilantin) therapy. J Rheumatol 7:733–736PubMedGoogle Scholar
  6. 6.
    Hashimoto Y, Takahashi H, Matsuo S, et al. (1996) Polymerase chain reaction of Borrelia burgdorferi flagellin gene in Shulman syndrome. Dermatology 192:136–139PubMedCrossRefGoogle Scholar
  7. 7.
    Granter SR, Barnhill RL, Duray PH (1996) Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol 18:465–473PubMedCrossRefGoogle Scholar
  8. 8.
    Hayashi N, Igarashi A, Matsuyama T, et al. (2000) Eosinophilic fasciitis following exposure to trichloroethylene: successful treatment with cyclosporine. Br J Dermatol 142:830–832PubMedCrossRefGoogle Scholar
  9. 9.
    Liou CH, Huang GS, Taylor JA, et al. (2003) Eosinophilic fasciitis in a military recruit: MRI evaluation with clinical correlation. Skeletal Radiol 32:52–57PubMedCrossRefGoogle Scholar
  10. 10.
    Littlejohn GO, Keystone EC (1980) Eosinophilic fasciitis and aplastic anemia. J Rheumatol 7:730–732PubMedGoogle Scholar
  11. 11.
    Khanna D, Verity A, Grossman JM (2002) Eosinophilic fasciitis with multiple myeloma: a new hematological association. Ann Rheum Dis 61:1111–1112PubMedCrossRefGoogle Scholar
  12. 12.
    Michaels RM (1982) Eosinophilic fasciitis complicated by Hodgkin’s disease. J Rheumatol 9:473–476PubMedGoogle Scholar
  13. 13.
    Ohno M, Nagaoka S, Onari K, et al. (2001) Remitting fasciitis without eosinophilia: a new disease entity? A case report. Rheumatology (Oxford) 40:1428–1430CrossRefGoogle Scholar
  14. 14.
    Antic M, Lautenschlager S, Itin PH (2006) Eosinophilic fasciitis 30 years after—What do we really know? Report of 11 patients and review of the literature. Dermatology 213:93–101PubMedCrossRefGoogle Scholar
  15. 15.
    Kazatchkine MD, Bayry J, Lacroix-Desmazes S, et al. (2004) Mechanisms of action of intravenous immunoglobulin in autoimmune and inflammatory diseases. In: MC Dalakas, PJ Späth (ed) Intravenous immunoglobulins in the third millennium. The Parthenon Publishing Group, New York, pp 106–107Google Scholar
  16. 16.
    Agnew KL, Blunt D, Francis ND, et al. (2005) Magnetic resonance imaging in eosinophilic fasciitis. Clin Exp Dermatol 30:435–436PubMedCrossRefGoogle Scholar
  17. 17.
    Sugimoto T, Nitta N, Kashiwag A (2007) Usefulness of magnetic resonance imaging in eosinophilic fasciitis. Rheumatol Int 27:791–792PubMedCrossRefGoogle Scholar
  18. 18.
    Gotlib J, Cools J, Malone JM III, et al. (2004) The FIP1L1- PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia, implications for diagnosis, classification, and management. Blood 103:2879–2891PubMedCrossRefGoogle Scholar
  19. 19.
    Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 348(13):1201–1214Google Scholar
  20. 20.
    Grobner T (2006) Gadolinium: a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? Nephrol Dial Transplant 21:1104–1108PubMedCrossRefGoogle Scholar
  21. 21.
    Galan A, Cowper SE, Bucala R (2006) Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol 18:614–617PubMedCrossRefGoogle Scholar
  22. 22.
    Pouplin S, Daragon A, Le Loët X, et al. (1998) Treatment of eosinophilic fasciitis with methotrexate. J Rheumatol 25:606–607PubMedGoogle Scholar
  23. 23.
    Jensen E, Hess B, Hunziker T, et al. (2000) Eosinophilic fasciitis (Shulman syndrome). Schweiz Med Wochenschr 130:156–160PubMedGoogle Scholar
  24. 24.
    Bukiej A, Dropińsky J, Dyduch G, et al. (2005) Eosinophilic fasciitis successfully treated with cyclosporine, case report. Clin Rheumatol 24:634–636PubMedCrossRefGoogle Scholar
  25. 25.
    Valencia IC, Chang A, Kirsner RS, et al. (1999) Eosinophilic fasciitis responsive to treatment with pulse steroids and cyclosporine. Int J Dermatol 38:369–372CrossRefGoogle Scholar
  26. 26.
    Schiener R, Behrens-Williams SC, Gottlöber P, et al. (2000) Eosinophilic fasciitis treated with psoralen-ultraviolet A bath photochemotherapy. Br J Dermatol 142:804–807PubMedCrossRefGoogle Scholar
  27. 27.
    Solomon G, Barland P, Rifkin H (1982) Eosinophilic fasciitis responsive to cimetidine. Ann Intern Med 97:547–549PubMedGoogle Scholar

Copyright information

© Hospital for Special Surgery 2007

Authors and Affiliations

  • Virginia Florina Haiduc
    • 1
  • Doruk Erkan
    • 1
    • 5
    Email author
  • Kyriakos Kirou
    • 1
  • Sherri Birchansky
    • 2
  • Josephine Park
    • 3
  • Moris J. Danon
    • 4
  1. 1.Division of RheumatologyHospital for Special SurgeryNew YorkUSA
  2. 2.Department of Radiology and ImagingHospital for Special SurgeryNew YorkUSA
  3. 3.Joint Mobility Center, Department of RehabilitationHospital for Special SurgeryNew YorkUSA
  4. 4.Department of NeurologyHospital for Special SurgeryNew YorkUSA
  5. 5.Barbara Volcker CenterHospital for Special SurgeryNew YorkUSA

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