J Rheum Dis.  2011 Dec;18(4):306-310. 10.4078/jrd.2011.18.4.306.

A Case of Steroid and Methotrexate-Resistant Eosinophilic Fasciitis Treated with Adalimumab

• Lee SS
• Kim HO
• Na JB
• Lee JS
• Suh YS
• Cheon YH
• Kang MG
• Kim YE
• Lee SI

• KMID: 2223136
• DOI: http://doi.org/10.4078/jrd.2011.18.4.306

Abstract

Eosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inEosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inhibitor in EF following a steroid-resistant disease course. However, there has been no report on the experience in treatment of EF with a TNF alpha inhibitor in Korea. Hence, we report a case of steroid and methotrexate-resistant EF which was successfully treated with adalimumab, along with a review of the relevant articles.

Keyword

Eosinophilic fasciitis; Glucocorticoid; TNF alpha inhibitor

MeSH Terms

Antibodies, Monoclonal, Humanized
Eosinophilia
Eosinophils
Extremities
Fascia
Fasciitis
Glucocorticoids
Immunosuppressive Agents
Inflammation
Korea
Sclerosis
Adalimumab
Antibodies, Monoclonal, Humanized
Eosinophilia
Fasciitis
Glucocorticoids
Immunosuppressive Agents

Figure

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  Figure 1. (A) The initial MRI shows enhancement in the posterior layers of the thoracolumbar fascia (arrow). (B) The followup MRI shows an increase in the enhancement in the posterior layers of the thoracolumbar fascia. (C) After treatment with anti TNF-α agent, the MRI shows marked improvement in fasciitis.

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  Figure 2. Superficial fascia taken from the left vastus intermedius shows dense fibrous tissue with inflammatory cell infiltration in the thickened fascia (H&E stain, ×100).

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  Figure 3. Changes in the eosinophil count (%) during treatment.

Reference

References

1. Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians. 1975; 88:70–86.

2. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988; 17:221–31.

3. Helfman T, Falanga V. Eosinophilic fasciitis. Clin Dermatol. 1994; 12:449–55.

4. Baumann F, Brühlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005; 184:169–74.

5. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979; 96:493–518.

6. Pouplin S, Daragon A, Le Loët X. Treatment of eosinophilic fasciitis with methotrexate. J Rheumatol. 1998; 25:606–7.

7. Bukiej A, Dropiński J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. 2005; 24:634–6.

8. Whang YJ, Kim KM, Oh SJ, Jang KY, Lee SI, Yoo WH, et al. Diagnostic availability of ultrasonography and the effects of methotrexate therapy in patients with eosinophilic fasciitis: report of two cases. J Korean Rheum Assoc. 2006; 13:56–63.

9. 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; 49:1184–8.

10. 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; 32:52–7.

11. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979; 96:493–518.

12. 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; 47:29–35.

13. Tzaribachev N, Holzer U, Schedel J, Maier V, Klein R, Kuemmerle-Deschner J. Infliximab effective in ste-roid-dependent juvenile eosinophilic fasciitis. Rheumatology (Oxford). 2008; 47:930–2.

14. Lam GK, Hummers LK, Woods A, Wigley FM. Efficacy and safety of etanercept in the treatment of scleroderma- associated joint disease. J Rheumatol. 2007; 34:1636–7.