Case Study 22 Shulmans Syndrome

  • 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].

  • Chun JH, Lee KH, Sung MS, Park CJ. Two cases of eosinophilic fasciitis. Ann Dermatol. 2011 Feb. 23(1):81-4. [Medline]. [Full Text].

  • 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].

  • Allen SC. Eosinophilic fasciitis in an African--possible benefit of chloroquine treatment. Postgrad Med J. 1984 Oct. 60(708):685-6. [Medline]. [Full Text].

  • 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].

  • Abstract

    Objective. To analyse therapeutic management of eosinophilic fasciitis (EF).

    Methods. We reviewed 34 adult patients with biopsy-proven EF. Analyses focused on the therapeutic management, including treatment modalities, responses and associated or predictive factors.

    Results. Thirty-four patients were included with a diagnosis age of 53 (15) years. They were featured by cutaneous manifestations (88%) including morphoea (41%), myalgia (86%) and hypereosinophilia (85%). Thirty-two patients (94%) were eligible for treatment evaluation and all received CSs as a first-line therapy. Fifteen patients (47%) received methylprednisolone pulses (MPPs) at treatment initiation and 14 patients (44%) received an immunosuppressive drug (ISD), usually MTX (86%), as a second-line therapy. Complete remission was achieved for 69% of patients, remission with disability 19% and failure 12%. A poor outcome was associated with a diagnosis time delay of >6 months [odds ratio (OR) = 14.7] and the lack of MPPs (OR = 12.9).

    Conclusion. Our study reports new insights into the therapeutic management of EF: (i) CS treatment remains the standard therapy for EF, taken alone or in association with an ISD; (ii) MPPs at initiation of treatment are associated with a better outcome and a lower need of ISD use; (iii) an ISD, usually MTX, might be useful as a second-line therapy, mainly in patients with morphoea-like lesions. Naturally, these practical conclusions should be confirmed by a prospective and multicentre study.

    fasciitis, eosinophilia, treatment, methotrexate, morphoea

    Introduction

    In 1975, Shulman described eosinophilic fasciitis (EF) as a rare connective tissue disease characterized by a symmetrical and painful swelling with a progressive induration of the skin and soft tissues [1, 2]. In the absence of international diagnostic criteria, diagnosis of EF is now based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils [3, 4]. Peripheral eosinophilia is present in 63–93% of patients, although not mandatory for EF diagnosis [3, 4].

    Therapeutic management of EF is one of the most significant challenges, as the clinical, biological and pathological features are well defined. Mainstay therapy is based on steroids with a partial or complete response in just over 60% of patients [4]. Use of immunosuppressive drugs (AZA, CYC, MTX, ciclosporin and, more recently, anti-TNF-α and rituximab) is not codified, and is mostly justified by failure of, or dependence on, high doses of CSs [4–11].

    The aim of our study was to highlight new insights into EF therapeutic management. We set up a retrospective study of 34 adult patients with biopsy-proven EF and analysed their therapeutic management, including treatment modalities, response and associated prognostic factors.

    Methods

    Study design

    We retrospectively reviewed medical charts of adult patients treated or referred to our University Hospital for EF between January 1992 and May 2010. EF diagnosis was based on suggestive clinical and laboratory findings and a biopsy-proven fascia involvement [1, 4, 5]. No patient had an l-tryptophane-associated eosinophilia–myalgia syndrome. Informed consent was obtained for each patient.

    Data collection and variables of interest

    Collected data included demographic parameters, presence, type and distribution of cutaneous, articular and muscular involvement. Morphoea was diagnosed clinically when skin sclerosis was noticed and histologically when a dermal fibrosis was noticed [4, 12]. Subcutis sclerosis was diagnosed clinically when a deep subcutis induration was present without involvement of the superficial skin. Baseline biological data included eosinophil count, creatinine phosphokinase (CPK), CRP, serum gammaglobulin level and, when available, immunological markers: ANCA, ANA, anti-ENA antibodies. Peripheral hypereosinophilia was defined by an absolute number of peripheral eosinophils >500/mm3 [3].

    Skin–fascio–muscular biopsy

    All patients underwent a full thickness skin to muscle biopsy following a standard protocol and the pathological analyses were performed in the same laboratory by M.T. or D.O. Diagnosis of EF was assessed by the evidence of a fasciitis with a thickened fascia and inflammatory infiltrates composed of lymphocytes and/or eosinophils [4].

    Treatment

    Data regarding treatment regimen, clinical and biological responses were collected. Recorded treatments included oral steroids, i.v. methylprednisolone pulses (MPPs), immunosuppressive drugs (ISDs) (MTX, AZA) and immunomodulatory drugs (HCQ, colchicine). Prescribed treatment was the responsibility of the patient's physician.

    Treatment response definitions

    Response to treatment was defined as: (i) failure (patients with persistent active physical signs and symptoms); (ii) remission (patients with disability, i.e. persistent joint contractures, tendon retraction or subcutis sclerosis); and (iii) complete remission (free of symptoms at end of follow-up and resolution of physical findings). A poor outcome was defined as failure or presence of a disability. A complete laboratory response was defined as the normalization of peripheral eosinophil count.

    Statistical analysis

    Continuous variables were compared using the t-test or the Kruskal–Wallis test, and categorical variables using Fisher's exact or χ2 tests as appropriate. A stepwise multiple logistic regression analysis was used to assess independent associations based on the results of univariate analyses (P < 0.1). Rates of event-free survival (ISD, poor outcome) over time were plotted by Kaplan–Meier's method and compared using the log-rank test. A Cox proportional hazards multiple regression model was used to evaluate the relative risk (RR) associated with the introduction of an ISD. All tests were two-tailed and a P ≤ 0.05 was considered statistically significant. All statistical analyses were performed using the MedCalc software version 11.1.1.0 (Mariakerke, Belgium).

    Results

    Patients characteristics and clinical manifestations

    Thirty-four patients were included (Table 1). At diagnosis, a cutaneous involvement was present in 30 (88%) patients and upper extremities were affected in all cases. Eighteen (53%) patients had a groove sign on forearm (Fig. 1A) and 14 (41%) patients exhibited morphoea-like lesions (Fig. 1B). Thirteen (38%) patients had arthralgia and 29 (86%) complained of muscle pain involving upper (86%) and lower extremities (59%). Nine (26%) patients experienced weight loss and 13 (38%) patients asthenia, but none had fever.

    Fig. 1

    Cutaneous findings and outcome. (A) Typical groove sign (depressed veins aspect) of the left forearm of a patient with EF. (B) A morphoea-like skin lesion on the back of a patient with EF. (C and D) Kaplan–Meier curve analysis of the effect of the presence of morphoea (C), the lack of MPPs at treatment initiation (D) on the probability of receiving an ISD as a second-line therapy.

    0 Replies to “Case Study 22 Shulmans Syndrome”

    Lascia un Commento

    L'indirizzo email non verrà pubblicato. I campi obbligatori sono contrassegnati *