4. Laboratory analysis

Blood workup in patients with EF usually reveals peripheral eosinophilia (>7%), elevated ESR, and hypergammaglobulinemia. However, because of poor correlation between the degree of eosinophilia and disease severity, the laboratory analysis using this parameter is not a very accurate predictor for monitoring disease course and activity.2

5. Etiology

Review of the literature suggests several etiologic factors contributing to the development of EF with no established consensus among these reports. The most frequently reported factors are physical exertion, trauma, ingestion of L-tryptophan, statins, arthropod bite, and borrelliosis.9-11


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6. Treatment

High-dose systemic corticosteroid therapy is the mainstay of treatment for EF.2 Other drugs, such as cyclosporine, hydroxychloroquine, cimetidine, azathioprine, and D-penicillamine, have been used to treat EF with varying success.12,13

7. Discussion

Eosinophilic fasciitis is a rare form of sclerotic disease that has been considered a part scleroderma and systemic sclerosis. However, EF is not associated with Reynaud’s phenomenon, and internal organs are not involved (as is commonly seen in sclerosis and scleroderma). Most patients present with painful swelling and indurations of skin, primarily involving proximal limb musculature. Joint contracture may occur as a sequel of indurations and sclerosis of the subcutaneous tissue. Lab analysis shows eosinophilia >7% in the differential blood cell count and elevated CRP and ESR. The cause of EF is not known, but strenuous exercise and ingestion of L-tryptophan have been considered as predisposing factors for the development of this disease. EF has also been associated with the use of simvastatin. While the identification of EF is made by clinical presentation, the final diagnosis is established by full-thickness skin-to-muscle biopsy.

In our patient, typical symmetrical sclerosis and indurations of both upper arms and eosinophilia >7% was found. High doses of prednisone (2mg/kg/day) resulted in dramatic clinical improvement within three weeks, which is in agreement with previous studies.2,14

Studies have also shown a favorable outcome using the combination of corticosteroid-hydroxychloroquine.2 Because EF has been regarded as a variant of morphea, phototherapy utilizing UVA plus psoralen (PUVA) has also been used as treatment.15 However, the exact mechanism of action of PUVA and long-term safety in patients with EF is not known. Individuals with EF have also been reported to respond well to methotrexate, cimetidine, penicillamine, and cyclosporine.12,13 Penicillamine and cyclosporine are especially useful when corticosteroids are less effective or contraindicated. The current consensus suggests that early therapy with systemic corticosteroids may be associated with a good outcome in EF patients.

Dr. Shailendra Saxena is assistant professor, department of family medicine, Creighton University Medical Center (CUMC), Omaha, Neb. Dr. Phulwani is a first-year resident in the department of surgery at CUMC. Dr. Shikhar Saxena is a first-year cardiology fellow at the University of Nebraska Medical Center in Omaha. Dr. Khatri is

a family physician in Omaha. Dr. Rafiq is assistant professor, department of family medicine, CUMC.

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, et al. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988;17:221-231.

3. Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after – what do we really know? Report of 11 patients and review of the literature. Dermatology. 2006;213:93-101.

4. 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.

5. Yamanishi Y, Ishioka S, Yamakido M. Complete remission of relapsing eosinophilic fasciitis associated with bronchial asthma following regular steroid inhalation. Rheumatology (Oxford). 2000;39:339-340. Available at rheumatology.oxfordjournals.org/cgi/content/full/39/3/339.

6. Khanna D, Verity A, Grossman JM. Eosinophilic fasciitis with multiple myeloma: A new haematological association. Ann Rheum Dis. 2002;61:1111-1112. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC1753970/.

7. Minciullo PL, Morabito F, Mandaglio R, et al. Eosinophilic fasciitis associated with autoimmune phenomena after bone marrow transplantation: report of two cases. Clin Rheumatol. 2006;25:80-82.

8. Baumann F, Bruhlmann P, Andreisek G, et al. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005;184:169-174. Available at www.ajronline.org/cgi/content/full/184/1/169.

9. Blauvelt A, Falanga V. Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination. Arch Dermatol. 1991;127:1159-1166.

10. Hamilton ME. Eosinophilic fasciitis associated with L-tryptophan ingestion. Ann Rheum Dis. 1991;50:55-56. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC1004327/.

11. Choquet-Kastylevsky G, Kanitakis J, Dumas V, et al. Eosinophilic fasciitis and simvastatin. Arch Intern Med. 2001;161:1456-1457.

12. Bukiej A, Dropinski J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. 2005;24:634-636.

13. Herson S, Brechignac S, Godeau P. Cimetidine in eosinophilic fasciitis. Ann Intern Med. 1990;113:412-413.

14. Britt WJ, Duray PH, Dahl MV, Goltz RW. Diffuse fasciitis with eosinophilia: a steroid-responsive variant of scleroderma. J Pediatr. 1980;97:432-434.

15. Schiener R, Behrens-Williams SC, Gottlober P, et al. Eosinophilic fasciitis treated with psoralen-ultraviolet A bath photochemotherapy. Br J Dermatol. 2000;142:804-807.

All electronic documents accessed August 15, 2010.