Eosinophilic Fasciitis Clinical Presentation
- Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD more...
Classically, patients with eosinophilic fasciitis (EF) present with symmetric swelling of the skin associated with an aching of the affected extremities, which may develop acutely over a period of days to weeks. Eosinophilic fasciitis may also manifest subacutely. In addition, if patients present later in their disease course, they are more likely to have symptoms of induration or fibrosis of the affected areas.
The onset of illness is not accompanied by fever or other systemic symptoms. In up to half of all patients, disease onset follows an episode of strenuous physical exercise or activity.
Neither Raynaud phenomenon nor symptoms of respiratory, gastrointestinal, or cardiac involvement are typically present.
Inflammatory arthritis has been reported and manifests as joint pain, swelling, and morning stiffness.[18, 20]
With progressive fibrosis, patients may endorse limited range of motion due to joint contractures and paresthesias in a distribution pattern consistent with carpal tunnel syndrome.
Cutaneous manifestations include the following[18, 20] :
The cutaneous manifestations of eosinophilic fasciitis evolve as the disease progresses. In the acute inflammatory stage, cutaneous changes include erythematous swelling and nonpitting edema. These findings are later replaced by skin induration, and, eventually, fibrosis predominates. The affected skin is taut and firmly adherent to underlying tissues. Dimpling, peau d'orange, and venous furrowing, or the "groove sign," can be seen. See the images below.The arm of this patient demonstrates the puckered, so-called orange-peel or cobblestone skin that may occur in eosinophilic fasciitis.The skin of the patient's back appears shiny due to the stretched dermis overlying an inflamed fascia. Mild diffuse hyperpigmentation is present, along with a U-shaped area of hypopigmentation extending approximately from T10 to L4.
Cutaneous manifestations are generally bilateral and symmetric. The upper extremity, proximal and distal to the elbow, and the lower extremity, proximal and distal to the knee, are most commonly involved. The trunk and neck can also be involved. Face and hand involvement are rare.
A concurrent localized lesion of morphea may be seen in 25% of patients.
Extracutaneous manifestations include the following:
Joint contractures represent the most common extracutaneous manifestation of eosinophilic fasciitis, occurring in 50%-75% of patients, and can affect elbows, wrists, ankles, knees, and shoulders. [18, 20] Extensive truncal fibrosis may limit chest expansion. A clawlike deformity of the hand has been described.
Inflammatory arthritis was reported in roughly 40% of patients in two series. [18, 20] The knees, wrists, hands, and feet appear to be most commonly involved.
Carpal tunnel syndrome is seen in 16%-23% of patients. [18, 20, 21]
Clinically significant visceral involvement is rare, limited to case reports. If present, significant visceral involvement should prompt investigation of an alternative diagnosis. When pursued, specific testing with pulmonary function testing, esophagogastroduodenoscopy (EGD), and electromyelography (EMG) may demonstrate subtle or nonspecific abnormalities. 
The etiology of eosinophilic fasciitis is unknown. The clinical manifestations of eosinophilic fasciitis are the result of an inflammatory response in the affected tissues. As explained above, our current understanding of eosinophilic fasciitis relies on a relatively few case series and case reports. As such, many etiologic factors have been suggested with varying degrees of supporting evidence. It may be possible that any of these factors, alone or in combination, could initiate this inflammatory response.
Several possible triggers have been reported with some consistency. A preceding history of vigorous exercise or trauma has been reported in 30%-50% of patients.[18, 20] Multiple drugs have also been implicated, including simvastatin, atorvastatin, and phenytoin.[23, 24, 25]
Several cases have demonstrated positive Borrelia serologies. The significance of this finding continues to be debated. Spirochetes were visualized by silver stain in 4 patients in one study. These findings have not been repeated. It has been suggested that positive serology for Borrelia represents an epiphenomenon among cases from Borrelia -endemic areas and is insufficient evidence of infection and therefore does not support a causal association.
Eosinophilic fasciitis shares clinical similarities, as well as key differences, with eosinophilia-myalgia syndrome. Some studies have suggested an association between l-tryptophan ingestion and eosinophilic fasciitis.[28, 29] Despite this, there is no consistent association between l-tryptophan or other dietary exposure and eosinophilic fasciitis. As evidence, l-tryptophan use was significantly associated with dyspnea, an uncommon finding in eosinophilic fasciitis cases. In another instance, a patient with eosinophilic fasciitis had used l-tryptophan for several years but had started a formal exercise program 2 weeks prior to disease onset.
Multiple additional etiologic triggers have been suggested by single or infrequent case reports.
As with etiology, eosinophilic fasciitis has been associated with several diseases.
Hematologic diseases have been consistently reported and are supported by large case series and case reports.[18, 20, 31] The spectrum of associated hematologic disease is broad and includes aplastic and hemolytic anemia, thrombocytopenia, myeloproliferative disorders, myelodysplastic disorders, lymphoma, leukemia, monoclonal gammopathy of undetermined significance (MGUS), and multiple myeloma.[31, 32, 33]
An association with thyroid disease has been reported in several cases. Eosinophilic fasciitis has rarely been linked to solid-organ tumors and primary biliary cirrhosis, in addition to several other diseases. These disease associations may suggest a shared pathophysiology of cellular dysregulation and/or autoimmunity.
Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome?. Trans Assoc Am Physicians. 1975. 88:70-86. [Medline].
Lebeaux D, Francès 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). 2011 Nov 25. [Medline].
Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol. 2007 Sep. 26(9):1445-51. [Medline].
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 Jan 1. 152 (1):97-9. [Medline].
Viallard JF, Taupin JL, Ranchin V, Leng B, Pellegrin JL, Moreau JF. Analysis of leukemia inhibitory factor, type 1 and type 2 cytokine production in patients with eosinophilic fasciitis. J Rheumatol. 2001 Jan. 28(1):75-80. [Medline].
Dziadzio L, Kelly EA, Panzer SE, Jarjour N, Huttenlocher A. Cytokine abnormalities in a patient with eosinophilic fasciitis. Ann Allergy Asthma Immunol. 2003 Apr. 90(4):452-5. [Medline].
Toquet C, Hamidou MA, Renaudin K, Jarry A, Foulc P, Barbarot S. In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis. J Rheumatol. 2003 Aug. 30(8):1811-5. [Medline].
Kahari VM, Heino J, Niskanen L, et al. Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol. 1990 May. 126(5):613-7. [Medline].
Peltonen J, Kahari L, Jaakkola S, et al. Evaluation of transforming growth factor beta and type I procollagen gene expression in fibrotic skin diseases by in situ hybridization. J Invest Dermatol. 1990 Mar. 94(3):365-71. [Medline].
Mori Y, Kahari VM, Varga J. Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep. 2002 Apr. 4(2):113-22. [Medline].
Jinnin M, Ihn H, Yamane K, Asano Y, Yazawa N, Tamaki K. Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis. Br J Dermatol. 2004 Aug. 151(2):407-12. [Medline].
Moutsopoulos HM, Webber BL, Pavlidis NA, Fostiropoulos G, Goules D, Shulman LE. Diffuse fasciitis with eosinophilia. A clinicopathologic study. Am J Med. 1980 May. 68(5):701-9. [Medline].
Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979 Aug. 96(2):493-518. [Medline].
Brent LH, Abruzzo JL. Localized eosinophilic fasciitis in a patient with rheumatoid arthritis. J Rheumatol. 1985 Oct. 12(5):987-9. [Medline].
Nawata Y, Sueishi M, Koike T, Tomioka H. Eosinophilic fasciitis with autoimmune features. Arthritis Rheum. 1983 May. 26(5):688. [Medline].
Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988 May. 17(4):221-31. [Medline].
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(2):93-101. [Medline].
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 Jan. 47(1):29-35. [Medline].
Wollheim FA, Lindstrom CG, Eiken O. Eosinophilic fasciitis complicated by carpal tunnel syndrome. J Rheumatol. 1981 Sep-Oct. 8(5):856-60. [Medline].
Caspi D, Fishel R, Varon M, et al. Multisystem presentation of eosinophilic fasciitis. Rheumatol Rehabil. 1982 Nov. 21(4):218-21. [Medline].
Choquet-Kastylevsky G, Kanitakis J, Dumas V, Descotes J, Faure M, Claudy A. Eosinophilic fasciitis and simvastatin. Arch Intern Med. 2001 Jun 11. 161(11):1456-7. [Medline].
DeGiovanni C, Chard M, Woollons A. Eosinophilic fasciitis secondary to treatment with atorvastatin. Clin Exp Dermatol. 2006 Jan. 31(1):131-2. [Medline].
Buchanan RR, Gordon DA, Muckle TJ, McKenna F, Kraag G. The eosinophilic fasciitis syndrome after phenytoin (dilantin) therapy. J Rheumatol. 1980 Sep-Oct. 7(5):733-6. [Medline].
Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol. 1996 Oct. 18(5):465-73. [Medline].
Antón E. Failure to demonstrate Borrelia burgdorferi-specific DNA in lesions of eosinophilic fasciitis. Histopathology. 2006 Jul. 49(1):88-90. [Medline].
Blauvelt A, Falanga V. Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination. Arch Dermatol. 1991 Aug. 127(8):1159-66. [Medline].
Hibbs JR, Mittleman B, Hill P, Medsger TA Jr. L-tryptophan-associated eosinophilic fasciitis prior to the 1989 eosinophilia-myalgia syndrome outbreak. Arthritis Rheum. 1992 Mar. 35(3):299-303. [Medline].
Lee P. Eosinophilic fasciitis: new associations and current perspectives [editorial]. J Rheumatol. 1981 Jan-Feb. 8(1):6-8. [Medline].
Doyle JA, Connolly SM, Hoagland HC. Hematologic disease in scleroderma syndromes. Acta Derm Venereol. 1985. 65(6):521-5. [Medline].
Masuoka H, Kikuchi K, Takahashi S, Kakinuma T, Hayashi N, Furue M. Eosinophilic fasciitis associated with low-grade T-cell lymphoma. Br J Dermatol. 1998 Nov. 139(5):928-30. [Medline].
Garcia VP, de Quiros JF, Caminal L. Autoimmune hemolytic anemia associated with eosinophilic fasciitis. J Rheumatol. 1998 Sep. 25(9):1864-5. [Medline].
Hur JW, Lee HS, Uhm WS, et al. Eosinophilic fasciitis associated with autoimmune thyroiditis. Korean J Intern Med. 2005 Jun. 20(2):180-2. [Medline].
Katz JD, Wakem CJ, Parke AL. L-tryptophan associated eosinophilia-myalgia syndrome. J Rheumatol. 1990 Nov. 17(11):1559-61. [Medline].
Shulman LE. The eosinophilia-myalgia syndrome associated with ingestion of L- tryptophan. Arthritis Rheum. 1990 Jul. 33(7):913-7. [Medline].
Kaufman LD, Krupp LB. Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia. Curr Opin Rheumatol. 1995 Nov. 7(6):560-7. [Medline].
Varga J, Kähäri VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. 1997 Nov. 9(6):562-70. [Medline].
Abeles M, Belin DC, Zurier RB. Eosinophilic fasciitis: a clinicopathologic study. Arch Intern Med. 1979 May. 139(5):586-8. [Medline].
Falanga V, Medsger TA Jr. Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis. J Am Acad Dermatol. 1987 Oct. 17(4):648-56. [Medline].
Kim SW, Rice L, Champlin R, Udden MM. Aplastic anemia in eosinophilic fasciitis: responses to immunosuppression and marrow transplantation. Haematologia (Budap). 1997. 28(3):131-7. [Medline].
Naschitz JE, Yeshurun D, Zuckerman E, Rosenbaum M, Misselevitch I, Shajrawi I. Cancer-associated fasciitis panniculitis. Cancer. 1994 Jan 1. 73(1):231-5. [Medline].
Sugimoto T, Nitta N, Kashiwagi A. Usefulness of magnetic resonance imaging in eosinophilic fasciitis. Rheumatol Int. 2007 Jun. 27(8):791-2. [Medline].
Moulton SJ, Kransdorf MJ, Ginsburg WW, Abril A, Persellin S. Eosinophilic fasciitis: spectrum of MRI findings. AJR Am J Roentgenol. 2005 Mar. 184(3):975-8. [Medline].
Agnew KL, Blunt D, Francis ND, Bunker CB. Magnetic resonance imaging in eosinophilic fasciitis. Clin Exp Dermatol. 2005 Jul. 30(4):435-6. [Medline].
Baumann F, Bruhlmann P, Andreisek G, et al. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005 Jan. 184(1):169-74. [Medline].
Dybowski F, Neuen-Jacob E, Braun J. Eosinophilic fasciitis and myositis: use of imaging modalities for diagnosis and monitoring. Ann Rheum Dis. 2008 Apr. 67(4):572-4. [Medline].
Kissin EY, Garg A, Grayson PC, Dubreuil M, Vradii D, York M, et al. Ultrasound assessment of subcutaneous compressibility: a potential adjunctive diagnostic tool in eosinophilic fasciitis. J Clin Rheumatol. 2013 Oct. 19(7):382-5. [Medline].
Bertken R, Shaller D. Chronic progressive eosinophilic fasciitis: report of a 20-year failure to attain remission. Ann Rheum Dis. 1983 Feb. 42(1):103-5. [Medline].
Carneiro S, Brotas A, Lamy F, et al. Eosinophilic fasciitis (Shulman syndrome). Cutis. 2005 Apr. 75(4):228-32. [Medline].
Daniel RS, Brown AN. Case report of unilateral eosinophilic fasciitis in a Vietnamese woman. Am J Med Sci. 2009 Feb. 337(2):153-4. [Medline].
Cramer SF, Kent L, Abramowsky C, Moskowitz RW. Eosinophilic fasciitis. Immunopathology, ultrastructure, literature review,a nd consideration of its pathogenesis and relation to scleroderma. Arch Pathol Lab Med. 1982 Feb. 106(2):85-91. [Medline].
Kähäri VM, Heino J, Niskanen L, Fräki J, Uitto J. Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol. 1990 May. 126(5):613-7. [Medline].
Manzini C, Sebastiani M, Giuggioli D, Manfredi A, Colaci M, Cesinaro A, et al. D-penicillamine in the treatment of eosinophilic fasciitis: case reports and review of the literature. Clin Rheumatol. 2011 Oct 12. [Medline].
Suzuki G, Itoh Y, Horiuchi Y. Surgical management of eosinophilic fasciitis of the upper extremity. J Hand Surg Br. 1997 Jun. 22(3):405-7. [Medline].
Chan MK, Lages W. Eosinophilic fasciitis: visceral involvement. Arch Intern Med. 1982 Nov. 142(12):2201-2. [Medline].
Tzaribachev N, Holzer U, Schedel J, Maier V, Klein R, Kuemmerle-Deschner J. Infliximab effective in steroid-dependent juvenile eosinophilic fasciitis. Rheumatology (Oxford). 2008 Jun. 47(6):930-2. [Medline].
Tahara K, Yukawa S, Shoji A, Hayashi H, Tsuboi N. Long-term remission by cyclosporine in a patient with eosinophilic fasciitis associated with primary biliary cirrhosis. Clin Rheumatol. 2008 Sep. 27(9):1199-201. [Medline].
Herson S, Brechignac S, Godeau P. Cimetidine in eosinophilic fasciitis. Ann Intern Med. 1990 Sep 1. 113(5):412-3. [Medline].
Bukiej A, Dropinski J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. 2005 Nov. 24(6):634-6. [Medline].
Lebeaux D, Francès 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 Mar. 51(3):557-61. [Medline].
de Masson A, Bouaziz JD, Peffault de Latour R, Benhamou Y, Moluçon-Chabrot C, Bay JO, et al. Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature. Medicine (Baltimore). 2013 Mar. 92(2):69-81. [Medline].