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Abstract
Systemic sclerosis (systemic scleroderma or SSc) is a chronic connective tissue disease of unknown etiology that causes widespread micro vascular damage and excessive deposition of collagen in the skin and internal organs, in particular GI tract, lung, heart, and kidney. We report the case of a 55 year old female patient who came to RIMS-GH, Kadapa complaints of cough, difficulty in swallowing, breathlessness since 2 weeks along with numbness of fingers. The patient had an ulcer on the right leg with white crystals; it shows the presence of calcinosis. Radiographic data showed bony erosion of the terminal phalanges and esophageal involvement, these findings indicates CREST syndrome. The biopsy report revealed it as scleroderma. The anti-nuclear antibodies and anti-topoisomerase I antibody tests were confirmed as diffuse cutaneous scleroderma. This case illustrates the clinical manifestations of a diffuse cutaneous scleroderma with CREST syndrome features.
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References
- [1] John V. Systemic Sclerosis (Scleroderma) and Related Disorders in Harrison’s Principles of internal medicine. (Dan L, et al.,) 18th edt, 2012, McGraw-Hill publishers, Page no: 2757 – 2769.
- [2] Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum 1998; 41:778– 99.
- [3] Hinchcliff M, Varga J. Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician. 2008; 78(8): 961-8.
- [4] Winterbuaer R: Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly and subcutaneous calcinosis: A syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp 114: 361, 1964.
- [5] Moroi Y, Peebles C, Frizler M, Steingerwald J, Tan E: Autoantibody to centromere (kinetochore) in scleroderma sera. Proc Natl Acad Sci USA 77: 1627, 1980.
- [6] Ferri C, Bernini L, Cecchetti R, et al: Cutaneous and serologic subsets of systemic sclerosis. J Rheumatol 18: 1826, 1991.
- [7] Frank van den Hoogen, Dinesh Khanna, Jaap Fransen, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747-1755.
References
[1] John V. Systemic Sclerosis (Scleroderma) and Related Disorders in Harrison’s Principles of internal medicine. (Dan L, et al.,) 18th edt, 2012, McGraw-Hill publishers, Page no: 2757 – 2769.
[2] Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum 1998; 41:778– 99.
[3] Hinchcliff M, Varga J. Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician. 2008; 78(8): 961-8.
[4] Winterbuaer R: Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly and subcutaneous calcinosis: A syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp 114: 361, 1964.
[5] Moroi Y, Peebles C, Frizler M, Steingerwald J, Tan E: Autoantibody to centromere (kinetochore) in scleroderma sera. Proc Natl Acad Sci USA 77: 1627, 1980.
[6] Ferri C, Bernini L, Cecchetti R, et al: Cutaneous and serologic subsets of systemic sclerosis. J Rheumatol 18: 1826, 1991.
[7] Frank van den Hoogen, Dinesh Khanna, Jaap Fransen, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747-1755.