Eosinophilic fasciitis
Authors:
H. Konkolová
Authors‘ workplace:
Oddělení klinické farmakologie, FN Plzeň
Published in:
Čes. Revmatol., 32, 2024, No. 1, p. 25-32.
Category:
Case Report
Overview
Eosinophilic fasciitis (EF) is a rare systemic autoimmune disease of unknown etiology, which manifests with progressive stiffening and fibrosis of the skin, most commonly on the extremities. Late diagnosis can lead to contracture formation, progressive functional impairment, and disability. Delay in diagnosis is often due to confusion with the significantly more common systemic sclerosis. Glucocorticoids are the first-line treatment of choice, supplemented with methotrexate where appropriate. Early initiation of treatment significantly increases the chance of a good therapeutic response.
In this case report, we present a brief overview of the clinical manifestations, diagnosis, and treatment of EF and present the case of a 62-year-old woman who was admitted to our department. The patient had typical limb involvement with swelling and fibrosis of the skin, but her disease progressed uncharacteristically rapidly and the trunk was also affected. The trunk involvement caused limitation of breathing excursions, chest tightness, and shortness of breath. Laboratory evidence showed eosinophilia and antinuclear antibody positivity. The diagnosis of eosinophilic fasciitis was confirmed by biopsy. Due to early diagnosis and initiation of treatment, rapid improvement occurred within a week of hospitalization. After 4 months of treatment, the patient has only minimal skin involvement on her forearms.
Keywords:
Eosinophilia – eosinophilic fasciitis – scleroderma-like – thickened skin
Sources
1. 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(4): 221–231.
2. Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 1975; 88: 70.
3. Florell SR, Egan CA, Gregory MC, et al. Eosinohilic fasciitis occuring four weeks after the onset of dialysis in a renal failure patient. J Cutan Med Surg. 2001; 5: 33.
4. Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripherall eosinophilia associated with Borelliad infection. Am J Dermatopathol. 1996; 18: 465.
5. Hashimoto Y, Takahashi H, Matsuo S, et al. Polymerase chain reaction of Borrelia burgdorferi flagellin gene in Shulman syndrome. Dermatology 1996; 192: 136.
6. Mosconi S, Streit M, Brönimann M, Braathen L. Eosinophilic fasciitis (Shulman syndrome). Dermatology 2002; 205: 204.
7. Long H, Zhang G, Wang L, Lu Q. Eosinophilic SkinDisease: A Comprehensive Review. Clin Rev Allergy Immunol. 2016; 50: 189.
8. Le Tallec E, Lescoat A, Balleria A, et al. Eosinophilic Fasciitis Triggered by Nivolumab: A Remarkable Efficacy of the mTOR inhibitor Sirolimus. J Thorac Oncol. 2020; 15: e29.
9. Chan KK, Margo C, Shoushtari A, et al. Eosinophilic Fasciitis Following Checkpoint Inhibitor Therapy: Four Cases and a Review of Literature. Oncologist 2020; 25: 140.
10. Bachmeyer C, Monge M, Dhôte R, et al. Eosinophilic fasciitis following idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Hashimoto’s disease. Dermatology 1999; 199: 282.
11. Farrell AM, Ross JS, Bunker CB. Eosinophilic faciitis associated with autoimmune thyroid diseas and myelodysplasia treated with pulsed methylprednisolone and antihistamines. Br J Dermatol. 1999; 140: 1185.
12. Imren S, Tüzüner N, Yazici H. Eosinophilic fasciitis with thyroid disease. Clin Exp Rheumatol. 1988; 6: 96.
13. Boiesen M, Keiding LM, Thomsen K. Eosinophilic fasciitis. Report of a case with features of other autoimmune disease. Dermatologica 1983; 167: 147.
14. Smiley AM, Husain M, Indenbaum S. Eosinophilic fasciitis in association with thyroid disease: a report of three cases. J Rheumatol. 1980; 7: 871.
15. Wright NA, Mazori DR, Patel M, Merola JF, Femia AN, Vleugels RA. Epidemiology and Treatment of Eosinophilic Fasciitis: An Analysis of 63 Patients From 3 Tertiary Care Centers. JAMA Dermatol. 2016; 152(1): 97–99.
16. Lebeaux D, Frances C, Barete S, Wechsler B, Dubourg O, Renoux J, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford) 2012; 51(3): 557–561.
17. Ihn H. Eosinophilic fasciitis: From pathophysiology to treatment. Allergol Int. 2019; 68(4): 437–439.
18. Olejárová M, Stehlíková E, Bečvář R, Ciferská H, Velenská Z, Procházková D, Šimonová K. Eozinofilní fasciitida. Čes. Revmatol. 2013; 21(2): 86–92.
19. Mazori DR, Femia AN, Vleugels RA. Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment. Curr Rheumatol Rep. 2017; 19(12): 74.
20. Jinnin M, Yamamoto T, Asano Y, Ishikawa O, Sato S, et al. Diagnostic criteria, severity classification, and clinical guidlines of eosinophilic fasciitis. J Dermatol. 2018; 45: 881–890.
21. Minciullo PL, Morabito F, Mandaglio R, Iacopino P, Gangemi S. Eosinophilic fasciitis associated with autoimmune phenomena after bone marrow transplantation: report of two cases. Clin Rheumatol. 2006; 25(1): 80–82.
22. 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(1): 29–35.
23. Castanet J, Lacour JP, Perrin C, Taillan B, Dubois D, Ortonne JP. Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody. Dermatology 1994; 189(3): 304–307.
24. Pinal-Fernandez I, Selva-O’ Callaghan A, Grau JM. Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev. 2014; 13(4–5): 379–382.
25. Heidary N, Cheung W, Wang N, Kamino H, Franks AG Jr. Eosinophilic fasciitis/generalized morphea overlap. Dermatol Online J. 2009; 15(8): 2.
26. Gorcey L, Femia AN, Eastham AB, Gaviola G, Vleugels RA. The role of magnetic resonance imaging in the diagnosis of early eosinophilic fasciitis prior to the development of cutaneous findings. J Invest Dermatol. 2014; 134(Suppl 1): S15.
27. al-Shaikh A, Freeman C, Avruch L, McKendry RJ. Use of magnetic resonance imaging in diagnosing eosinophilic fasciitis. Report of two cases. Arthritis Rheum. 1994; 37(11): 1602–1608.
28. Grados D, Martínez-Morillo M, Tejera B, Olivé A. A comment on „from diagnosis to remission: place of MRI in eosinophilic fasciitis“. Clin Rheumatol. 2011; 30(3): 441–442.
29. Chan V, Soans B, Mathers D. Ultrasound and magnetic resonance imaging features in a patient with eosinophilic fasciitis. Australas Radiol. 2004; 48(3): 414–417.
30. Desvignes-Engelbert A, Saulière N, Loeuille D, Blum A, Chary-Valckenaere I. Polymyalgia revealing eosinophilic fasciitis in a young male: Contribution of magnetic resonance imaging. Joint Bone Spine 2010; 77(4): 367–368.
31. Cheriet S, Chastan M, Levesque H, Marie I. Positron emission tomography in the diagnosis of eosinophilic fasciitis. QJM 2011; 104(11): 987–988.
32. Kim HJ, Lee SW, Kim GJ, Lee JH. Usefulness of FDG PET/CT in the diagnosis of eosinophilic fasciitis. Clin Nucl Med. 2014; 39(9): 801–802.
33. Marie I, Sauvetre G. Fluorodeoxyglucose positron emission tomography in eosinophilic fasciitis. Joint Bone Spine 2014; 81(6): 541.
34. Kurimoto R, Ikeda K, Nakagomi D, Nakajima H. Eosinophilic Fasciitis Illustrated by [(18)F] FDG-PET/CT. Intern Med. 2016; 55(16): 2321–2322.
35. Kissin EY, Garg A, Grayson PC, Dubreuil M, Vradii D, York M, Simms RW. Ultrasound assessment of subcutaneous compressibility: a potential adjunctive diagnostic tool in eosinophilic fasciitis. J Clin Rheumatol. 2013; 19(7): 382–385.
36. Mondal S, Goswami RP, Sinha D, Ghosh A. Ultrasound is a useful adjunct in diagnosis of eosinophilic fasciitis. Rheumatology (Oxford) 2015; 54(11): 2041.
37. Helfgott SM, Varga J. Eosinophilic fascitiis. UpToDate, Axford JS (Ed), Wolters Kluwer 2023.
38. Loupasakis K, Derk CT. Eosinophilic fasciitis in a pediatric pa-
tient. J Clin Rheumatol. 2010; 16(3): 129–131.
39. Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y, Ishikawa O. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol 2007; 26(9): 1445–1451.
40. Farrington ML, Haas JE, Nazar-Stewart V, Mellins ED. Eosinophilic fasciitis in children frequently progresses to scleroderma-like cutaneous fibrosis. J Rheumatol. 1993; 20(1): 128–132.
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Dermatology & STDs Paediatric rheumatology RheumatologyArticle was published in
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