DOI: https://dx.doi.org/10.18203/2349-3933.ijam20210274
Published: 2021-01-27

Systemic sclerosis: a rare presentation

Elvi Aprillia Karamoy, Jason Raymond Hotama, Ketut Suryana, Novitasari .

Abstract


Systemic sclerosis or so called scleroderma is an uncommon autoimmune inflammatory and fibrotic connective tissue disease involving multiple organs. The etiology of systemic sclerosis is currently unknown and its pathogenesis is only partially understood. Skin thickening and Raynaud’s phenomenon are the most common symptoms. Although systemic sclerosis is uncommon, it is associated with high morbidity and mortality. In this report, we present a case of a-43-year-old man with the complaint of weakness, tightening of the skin over the fingers, tingling in the soles of feet, nausea and significant weight loss. Laboratory examinations revealed positive ANA test, but negative anti topoisomerase I (anti-Scl-70), and anticentromere antibody.

Keywords


Systemic sclerosis, Scleroderma, Sclerodactyly, Skin thickening, Autoimmune

Full Text:

PDF

References


Ingegnoli F, Ughi N, Mihai C. Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis. Best Pract Res Clin Rheumatol. 2018;32(2):223–40.

Sticherling M. Systemic sclerosis – the dermatological perspective. J Ger Soc Dermatol. 2019;17(7):716–28.

Kucharz EJ, Kopeć-Mȩdrek M. Systemic sclerosis sine scleroderma. Adv Clin Exp Med. 2017;26(5):875–80.

Denton CP, Khanna D. Systemic sclerosis. Lancet [Internet]. 2017;390(10103):1685–99.

Schinke S, Riemekasten G. Systemic sclerosis. Internist. 2019;60(12):1251–69.

Van den Hombergh WMT, Carreira PE, Knaapen-Hans HKA, van den Hoogen FHJ, Fransen J, Vonk MC. An easy prediction rule for diffuse cutaneous systemic sclerosis using only the timing and type of first

symptoms and auto-antibodies: Derivation and validation. Rheumatol. 2016;55(11):2023–32.

Johnson SR. New ACR EULAR guidelines for systemic sclerosis classification. Curr Rheumatol Rep. 2015;17(5):1–8.

Sobolewski P, Maślińska M, Wieczorek M, Łagun Z, Malewska A, Roszkiewicz M, et al. Systemic sclerosis - Multidisciplinary disease: Clinical features and treatment. Reumatologia. 2019;57(4):221–33.

Pearson DR, Werth VP, Pappas-Taffer L. Systemic sclerosis: Current concepts of skin and systemic manifestations. Clin Dermatol. 2018;36(4):459–74.

Horner KL, Raugi GJ, Wemple MA. CREST syndrome. eMedicine Dermatology. 2008; Available at: http://www.emedicine.medscape. com. Accessed on 20 March 2020.

Tangri N, Young BM. Soft-tissue infection and underlying calcinosis of CREST syndrome. Canad Medic Assoc J. 2006;175(9):1060–1.

Herrick AL. Review: Pathogenesis of Raynaud’s phenomenon. Rheumatology. 2005;44:587–96.

Sunderkotter C, Riemekasten G. Pathophysiology and clinical consequences of Raynaud’s phenomenon related to systemic sclerosis. Rheumatology. 2006;45:iii33.

Sheehan NJ. Dysphagia and other manifestations of oesophageal involvement in the musculoskeletal diseases. Rheumatology. 2008;47(6):746–52.

Pitrez EH, Bredemeier M, Xavier RM, Capobianco KG, Restelli VG, Vieira MV, et al. Oesophageal dysmotility in systemic sclerosis: comparison of HRCT and scintigraphy. Br J Radiol. 2006;79:719–24.

Jones J, Brenner C, Chinn R, Bunjer CB. Pictorial review: Radiological associations with dermatological disease. Br J Radiol. 2005;78:662–71.

Hu PQ, Fertig N, Medsger TA, Wright TM. Molecular Recognition Patterns of Serum Anti-DNA Topoisomerase I Antibody in Systemic Sclerosis. J Immunol. 2004;173(4):2834–41.

Lt. John Scott Donoughe, DO, MPAS, PA-C. Scleroderma: Quick Recertification Series. 2018;(10):53-4.

Wigley FM, Korn JH, Csuka ME, et al. Oral iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis: a multicenter, placebo-controlled, double-blind study. Arthritis Rheum. 1998;41(4):670-7.




Bookmark and Share