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Current Rheumatology Reviews


ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Case Report

An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes?

Author(s): Rehab Ali Ibrahim*, Nour El-Hoda Hussien Abdalla, Engy Amr Husssein Shabaan and Noha Bassiouny Hassan Mostafa

Volume 15, Issue 2, 2019

Page: [172 - 175] Pages: 4

DOI: 10.2174/1573397114666180808091621

Price: $65


Background: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmune disease. Many conditions mimic the presentation of SSc, especially skin thickening and fibrosis. One of these conditions is scleredema, a rare collagen and mucin deposition disorder which was found to be associated with diabetes mellitus, streptococcal infection or monoclonal gammopathy.

Case Presentation: A 55 years old female presented with insidious onset and progressive course of diffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both hands and feet of 6 years’ duration associated with arthralgia, dysphagia and dyspnea on exertion of 1- year duration. There was no history of Raynaud’s phenomenon. Erythrocyte sedimentation rate was 100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonary function tests showed restrictive pattern and high-resolution computed tomography showed interstitial lung fibrosis. Patient was not fulfilling the American collage of rheumatology/European League Against Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histological appearance of scleredema. Investigations were done for disease association with scleredema. The patient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixation and bone marrow biopsy were done, and the patient was diagnosed as scleredema associated with immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib, cyclophosphamide, and dexamethasone was started with marked clinical and hematological improvement.

Conclusion: Many conditions mimic SSc including scleredema, which may be the initial presentation of multiple myeloma. Rheumatologists and dermatologists should be able to recognize these conditions to provide the suitable management and follow-up for these patients.

Keywords: Scleroderma, scleredema, scleroderma mimics, multiple myeloma, monoclonal gammopathy, fibrosis.

Graphical Abstract
Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am 2008; 34(1): 199-220.
Nashel J, Steen V. Scleroderma mimics. Curr Rheumatol Rep 2012; 14(1): 39-46.
Morgan ND, Hummers LK. Scleroderma Mimickers. Curr Treatm Opt Rheumatol 2016; 2(1): 69-84.
Kokpol C, Rajatanavin N, Rattanakemakorn P. Successful treatment of scleredema diabeticorum by combining local PUVA and colchicine: A case report. Case Rep Dermatol 2012; 4(3): 265-8.
Wright RA, Bernie H. Scleredema adultorum of Buschke with upper esophageal involvement. Am J Gastroenterol 1982; 77(1): 9-11.
Livieri C, Monafo V, Bozzola M, Marni E. Buschke’s scleredema and carditis: a clinical case. Pediatr Med Chir 1982; 4(6): 695-7.
Paz RA, Badra RE, Martí HM, Maxit MJ. Systemic Buschke’s scleredema with cardiomyopathy, monoclonal IgG kappa gammopathy and amyloidosis. Case report with autopsy. Medicina (B Aires) 1998; 58(5 Pt 1): 501-3.
Reeder CB, Reece DE, Kukreti V, et al. Cyclophosphamide, bortezomib and dexamethasone induction for newly diagnosed multiple myeloma: high response rates in a phase II clinical trial. Leukemia 2009; 23(7): 1337-41.
Durie BG, Harousseau JL, Miguel JS, et al. International uniform response criteria for multiple myeloma. Leukemia 2006; 20(9): 1467-73.
Gajendra S, Gupta R, Gupta R, Kumar L. Coexistence of scleroderma with multiple myeloma: A rare association. BMJ Case Rep 2013; 2013: 1-3.
Czirjak L, Varju C. Clinical features of scleroderma-like disorders: A challenge for the rheumatologist. Curr Rheumatol Rev 2006; 2(4): 369-79.
Pujol JA, Bueno M, Fuertes MA, Gimenez H, Carapeto FJ. Improvement of scleredema associated with IgA multiple myeloma after chemotherapy. Clin Exp Dermatol 1995; 20(2): 149-52.
Ohta A, Uitto J, Oikarinen AI, et al. Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro. J Am Acad Dermatol 1987; 16(1 Pt 1): 96-107.
Rimon D, Lurie M, Storch S, et al. Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum. Arch Intern Med 1988; 148(3): 551-3.
Rongioletti F, Kaiser F, Cinotti E, et al. Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients. J Eur Acad Dermatol Venereol 2015; 29(12): 2399-404.
Dasgupta A, Wahed A. Clinical chemistry, immunology and laboratory quality control. 1st ed. 2014; pp. 391-406.
Katzmann JA, Willrich MA, Kohlhagen MC, et al. Monitoring IgA multiple myeloma: Immunoglobulin heavy/light chain assays. Clin Chem 2015; 61(2): 360-7.
Rota E, Nallino MG, Bainotti S, Formica M. Nephrogenic systemic fibrosis: An unusual scleroderma-like fibrosing disorder. Rheumatol Int 2010; 30(10): 1389-91.
Szturz P, Adam Z, Vašků V, et al. Complete remission of multiple myeloma associated scleredema after bortezomib-based treatment. Leuk Lymphoma 2013; 54(6): 1324-6.
Vigolo S, Zuckermann J, Bittencourt RI, Silla L, Pilger DA. Comparison of cyclophosphamide-thalidomide-dexamethasone to bortezomib-cyclophosphamide-dexamethasone as induction therapy for multiple myeloma patients in Brazil. Hematol Oncol Stem Cell Ther 2017; 10(3): 135-42.

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