Systemic Sclerosis Differential Diagnoses

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 18, 2012
 
 

Differential Diagnoses

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Bozena Dziankowska-Bartkowiak, MD, PhD  Consulting Staff, Department of Dermatology, University Hospital, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Anna Zalewska, MD, PhD  Professor of Dermatology and Venereology, Psychodermatology Department, Chair of Clinical Immunology and Microbiology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Anna Sysa-Jedrzejowska, MD, PhD  Head, Professor, Department of Dermatology and Venereology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

Mark W Cobb, MD  Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Sakkas LI. New developments in the pathogenesis of systemic sclerosis. Autoimmunity. Mar 2005;38(2):113-6. [Medline].

  2. Nguyen C, Berezne A, Baubet T, et al. Association of Gender with Clinical Expression, Quality of Life, Disability, and Depression and Anxiety in Patients with Systemic Sclerosis. PLoS One. Mar 9 2011;6(3):e17551. [Medline]. [Full Text].

  3. Malcarne VL, Hansdottir I, McKinney A. Medical signs and symptoms associated with disability, pain, and psychosocial adjustment in systemic sclerosis. J Rheumatol. Feb 2007;34(2):359-67. [Medline].

  4. Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. Oct 1986;115(4):393-401. [Medline].

  5. Spencer-Green G, Alter D, Welch HG. Test performance in systemic sclerosis: anti-centromere and anti-Scl-70 antibodies. Am J Med. Sep 1997;103(3):242-8. [Medline].

  6. Aozasa N, Asano Y, Ashida R, et al. Systemic sclerosis with an unusual rapid development of huge calcinosis (tumoral calcinosis). J Dermatol. Mar 2 2011;[Medline].

  7. Wielosz E, Borys O, Zychowska I, Majdan M. Gastrointestinal involvement in patients with systemic sclerosis. Pol Arch Med Wewn. Apr 2010;120(4):132-6. [Medline].

  8. Schieir O, Thombs BD, Hudson M, Boivin JF, Steele R, Bernatsky S, et al. Prevalence, severity, and clinical correlates of pain in patients with systemic sclerosis. Arthritis Care Res (Hoboken). Mar 2010;62(3):409-17. [Medline].

  9. Kawakami T, Tsutsumi Y, Soma Y. Limited cutaneous systemic sclerosis induced by paclitaxel in a patient with breast cancer. Arch Dermatol. Jan 2009;145(1):97-8. [Medline].

  10. Khoo JJ, Pratt EJ. Phaeochromocytoma mimicking scleroderma. Int J Endocrinol. 2011;2011:917453. [Medline]. [Full Text].

  11. Itoh M, Yanaba K, Kobayashi T, Nakagawa H. Taxane-induced scleroderma. Br J Dermatol. Feb 2007;156(2):363-7. [Medline].

  12. Muangchan C, Harding S, Khimdas S, Bonner A, Baron M, Pope J. C - reactive protein (CRP) is associated with high disease activity in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG). Arthritis Care Res (Hoboken). May 3 2012;[Medline].

  13. Waszczykowska E, Kukulski K, Sysa-Jedrzejowska A, Dziankowska-Bartkowiak B, Gierach D, Omulecki A. Evaluation of esophageal passage in selected connective tissue diseases. J Med. 1997;28(3-4):163-74. [Medline].

  14. de Groote P, Gressin V, Hachulla E, et al. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis. Jan 2008;67(1):31-6. [Medline].

  15. Kepez A, Akdogan A, Sade LE, et al. Detection of Subclinical Cardiac Involvement in Systemic Sclerosis by Echocardiographic Strain Imaging. Echocardiography. Feb 2008;25(2):191-197. [Medline].

  16. De Santis M, Bosello SL, Capoluongo E, Inzitari R, Peluso G, Lulli P, et al. A vascular endothelial growth factor deficiency characterises scleroderma lung disease. Ann Rheum Dis. Mar 27 2012;[Medline].

  17. Blaszczyk M, Jarzabek-Chorzelska M, Jablonska S, et al. Autoantibodies to nucleolar antigens in systemic scleroderma: clinical correlations. Br J Dermatol. Oct 1990;123(4):421-30. [Medline].

  18. Bruns M, Herrmann K, Haustein UF. Immunologic parameters in systemic sclerosis. Int J Dermatol. Jan 1994;33(1):25-32. [Medline].

  19. Van Praet JT, Smith V, Haspeslagh M, Degryse N, Elewaut D, De Keyser F. Histopathological cutaneous alterations in systemic sclerosis: a clinicopathological study. Arthritis Res Ther. Feb 28 2011;13(1):R35. [Medline].

  20. Ohtsuka T. Relation between elevated high-sensitivity C-reactive protein and anti-mitochondria antibody in patients with systemic sclerosis. J Dermatol. Feb 2008;35(2):70-5. [Medline].

  21. Van Praet JT, Van Steendam K, Smith V, et al. Specific anti-nuclear antibodies in systemic sclerosis patients with and without skin involvement: an extended methodological approach. Rheumatology (Oxford). Feb 17 2011;[Medline].

  22. 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. Apr 1998;41(4):670-7. [Medline].

  23. Krasagakis K, Dippel E, Ramaker J, Owsianowski M, Orfanos CE. Management of severe scleroderma with long-term extracorporeal photopheresis. Dermatology. 1998;196(3):309-15. [Medline].

  24. Seyger MM, van den Hoogen FH, van Vlijmen-Willems IM, van de Kerkhof PC, de Jong EM. Localized and systemic scleroderma show different histological responses to methotrexate therapy. J Pathol. Apr 2001;193(4):511-6. [Medline].

  25. Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A Prospective Observational Study of Mycophenolate Mofetil Treatment in Progressive Diffuse Cutaneous Systemic Sclerosis of Recent Onset. J Rheumatol. Apr 1 2012;[Medline].

  26. Hider SL, Woodhead M, Taylor PM, Bruce IN. Lung fibrosis in systemic sclerosis treated with a combination of ciclosporin and azathioprine. Clin Exp Rheumatol. Mar-Apr 2006;24(2):215. [Medline].

  27. Valentini G, Paone C, La Montagna G, et al. Low-dose intravenous cyclophosphamide in systemic sclerosis: an open prospective efficacy study in patients with early diffuse disease. Scand J Rheumatol. Jan-Feb 2006;35(1):35-8. [Medline].

  28. Varai G, Earle L, Jimenez SA, Steiner RM, Varga J. A pilot study of intermittent intravenous cyclophosphamide for the treatment of systemic sclerosis associated lung disease. J Rheumatol. Jul 1998;25(7):1325-9. [Medline].

  29. Furukawa S, Yasuda S, Amengual O, Horita T, Atsumi T, Koike T. Protective effect of pravastatin on vascular endothelium in patients with systemic sclerosis: a pilot study. Ann Rheum Dis. Aug 2006;65(8):1118-20. [Medline].

  30. Blagojevic J, Matucci-Cerinic M. Are statins useful for treating vascular involvement in systemic sclerosis?. Nat Clin Pract Rheumatol. Feb 2009;5(2):70-1. [Medline].

  31. Verrecchia F, Laboureau J, Verola O, et al. Skin involvement in scleroderma--where histological and clinical scores meet. Rheumatology (Oxford). May 2007;46(5):833-41. [Medline].

  32. Chung L, Fiorentino DF, Benbarak MJ, et al. Molecular framework for response to imatinib mesylate in systemic sclerosis. Arthritis Rheum. Jan 29 2009;60(2):584-591. [Medline].

  33. Pannu J, Asano Y, Nakerakanti S, et al. Smad1 pathway is activated in systemic sclerosis fibroblasts and is targeted by imatinib mesylate. Arthritis Rheum. Aug 2008;58(8):2528-37. [Medline].

  34. Riera R, Andrade LE, Souza AW, Kayser C, Yanagita ET, Trevisani VF. Lidocaine for systemic sclerosis: a double-blind randomized clinical trial. Orphanet J Rare Dis. Feb 7 2011;6:5. [Medline]. [Full Text].

  35. Scope A, Sadetzki S, Sidi Y, et al. Breast cancer and scleroderma. Skinmed. Jan-Feb 2006;5(1):18-24. [Medline].

  36. Mathai SC, Hummers LK, Champion HC, et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum. Feb 2009;60(2):569-77. [Medline].

  37. Hissaria P, Roberts-Thomson PJ, Lester S, Ahern MJ, Smith MD, Walker JG. Cigarette smoking in patients with systemic sclerosis - reduces overall survival. Arthritis Rheum. Mar 24 2011;[Medline].

  38. Mozzetta A, Antinone V, Alfani S, et al. Mental health in patients with systemic sclerosis: a controlled investigation. J Eur Acad Dermatol Venereol. Mar 2008;22(3):336-40. [Medline].

  39. Kwakkenbos L, van Lankveld WG, Vonk MC, Becker ES, van den Hoogen FH, van den Ende CH. Disease-related and psychosocial factors associated with depressive symptoms in patients with systemic sclerosis, including fear of progression and appearance self-esteem. J Psychosom Res. Mar 2012;72(3):199-204. [Medline].

  40. Artlett CM, Smith JB, Jimenez SA. New perspectives on the etiology of systemic sclerosis. Mol Med Today. Feb 1999;5(2):74-8. [Medline].

  41. Black CM. The aetiopathogenesis of systemic sclerosis. J Intern Med. Jul 1993;234(1):3-8. [Medline].

  42. Blaszczyk M, Jablonska S. Linear scleroderma en Coup de Sabre. Relationship with progressive facial hemiatrophy (PFH). Adv Exp Med Biol. 1999;455:101-4. [Medline].

  43. Blaszczyk M, Krysicka-Janiger K, Jablonska S. Primary atrophic profound linear scleroderma. Report of three cases. Dermatology. 2000;200(1):63-6. [Medline].

  44. Bunn CC, Denton CP, Shi-Wen X, Knight C, Black CM. Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol. Jan 1998;37(1):15-20. [Medline].

  45. Charles C, Clements P, Furst DE. Systemic sclerosis: hypothesis-driven treatment strategies. Lancet. May 20 2006;367(9523):1683-91. [Medline].

  46. Chitale S, Watson J, Herrick A. Autologous skin grafting--a limb-saving procedure in a patient with diffuse cutaneous systemic sclerosis. Rheumatology (Oxford). Mar 2008;47(3):379-380. [Medline].

  47. Ciurzynski M, Bienias P, Lichodziejewska B, et al. Non-invasive diagnostic and functional evaluation of cardiac involvement in patients with systemic sclerosis. Clin Rheumatol. Feb 7 2008;[Medline].

  48. Clements PJ, Furst DE. Systemic Sclerosis. Baltimore, Md: Williams & Wilkins; 1996.

  49. Connolly MK. Scleroderma. Dermatol Ther. 2001;14:81-94.

  50. De Luca A, Terrone C, Tirri E, Rossetti SR, Valentini G. Vesical telangiectasias as a cause of macroscopic hematuria in systemic sclerosis. Clin Exp Rheumatol. Jan-Feb 2001;19(1):93-4. [Medline].

  51. Denton CP, Humbert M, Rubin L, Black CM. Bosentan therapy for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis. Jun 22 2006;[Medline].

  52. Hara T, Ogawa F, Muroi E, et al. Anti-p53 Autoantibody in Systemic Sclerosis: Association with Limited Cutaneous Systemic Sclerosis. J Rheumatol. Jan 15 2008;[Medline].

  53. Haustein UF, Anderegg U. Pathophysiology of scleroderma: an update. J Eur Acad Dermatol Venereol. Jul 1998;11(1):1-8. [Medline].

  54. Hudson M, Steele R, Taillefer S, Baron M; Canadian Scleroderma Research. Quality of life in systemic sclerosis: Psychometric properties of the World Health Organization Disability Assessment Schedule II. Arthritis Rheum. Jan 31 2008;59(2):270-278. [Medline].

  55. Jablonska S, Blaszczyk M. Scleroderma overlap syndromes. Adv Exp Med Biol. 1999;455:85-92. [Medline].

  56. Jelaska A, Arakawa M, Broketa G, Korn JH. Heterogeneity of collagen synthesis in normal and systemic sclerosis skin fibroblasts. Increased proportion of high collagen-producing cells in systemic sclerosis fibroblasts. Arthritis Rheum. Aug 1996;39(8):1338-46. [Medline].

  57. Jun JB, Kuechle M, Harlan JM, Elkon KB. Fibroblast and endothelial apoptosis in systemic sclerosis. Curr Opin Rheumatol. Nov 2003;15(6):756-60. [Medline].

  58. Kalogerou A, Gelou E, Mountantonakis S, Settas L, Zafiriou E, Sakkas L. Early T cell activation in the skin from patients with systemic sclerosis. Ann Rheum Dis. Aug 2005;64(8):1233-5. [Medline].

  59. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. Feb 1988;15(2):202-5. [Medline].

  60. Louneva N, Huaman G, Fertala J, Jimenez SA. Inhibition of systemic sclerosis dermal fibroblast type I collagen production and gene expression by simvastatin. Arthritis Rheum. Mar 30 2006;54(4):1298-1308. [Medline].

  61. MacGregor AJ, Canavan R, Knight C, et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). Apr 2001;40(4):453-9. [Medline].

  62. Majewski S, Szymal E. Pathogenesis of scleroderma. Medipress Dermatol. 1996;1(3):2-6.

  63. Medsger TA Jr, Masi AT. Epidemiology of systemic sclerosis (scleroderma). Ann Intern Med. May 1971;74(5):714-21. [Medline].

  64. Meyer O. How useful are serum autoantibodies in the diagnosis and prognosis of systemic sclerosis?. Clin Rheumatol. 1998;17(3):179-80. [Medline].

  65. Misra R, Darton K, Jewkes RF, Black CM, Maini RN. Arthritis in scleroderma. Br J Rheumatol. Sep 1995;34(9):831-7. [Medline].

  66. Mitchell H, Bolster MB, LeRoy EC. Scleroderma and related conditions. Med Clin North Am. Jan 1997;81(1):129-49. [Medline].

  67. Nelson JL, Furst DE, Maloney S, et al. Microchimerism and HLA-compatible relationships of pregnancy in scleroderma. Lancet. Feb 21 1998;351(9102):559-62. [Medline].

  68. Ozturk MA, Benekli M, Altundag MK, Guler N. Squamous cell carcinoma of the skin associated with systemic sclerosis. Dermatol Surg. Jul 1998;24(7):777-9. [Medline].

  69. Sanchez O, Sitbon O, Jais X, Simonneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest. Jul 2006;130(1):182-9. [Medline].

  70. Schwartz J, Gonzalez J, Palangio M. Extracorporeal photochemotherapy in progressive systemic sclerosis: a follow-up study. Int J Dermatol. May 1997;36(5):380-5. [Medline].

  71. Simeon CP, Armadans L, Fonollosa V, et al. Survival prognostic factors and markers of morbidity in Spanish patients with systemic sclerosis. Ann Rheum Dis. Dec 1997;56(12):723-8. [Medline].

  72. Steen VD, Medsger TA Jr, Osial TA Jr. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med. May 1984;76(5):779-86. [Medline].

  73. Steen VD, Powell DL, Medsger TA Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. Feb 1988;31(2):196-203. [Medline].

  74. Stone JH, Wigley FM. Management of systemic sclerosis: the art and science. Semin Cutan Med Surg. Mar 1998;17(1):55-64. [Medline].

  75. Takehara K, Soma Y, Ishibashi Y. Early detection of scleroderma spectrum disorders in patients with Raynaud's phenomenon. Dermatologica. 1991;183(3):164-8. [Medline].

  76. Varga J. Editorial overview: Advancing research in systemic sclerosis: from bench to bedside and back. Curr Opin Rheumatol. Nov 2003;15(6):745-7. [Medline].

  77. Wranicz JK, Strzondala M, Zielinska M, et al. [Evaluation of early cardiovascular involvement in patients with systemic sclerosis]. Przegl Lek. 2000;57(7-8):389-92. [Medline].

  78. Wulffraat NM, Woo P, Rooney M, De Meer K, Kuis W. Atypical juvenile generalized scleroderma presenting as polyarthritis and failure to thrive. Br J Rheumatol. Feb 1998;37(2):222-7. [Medline].

  79. Yamamoto T, Takagawa S, Katayama I, Mizushima Y, Nishioka K. Effect of superoxide dismutase on bleomycin-induced dermal sclerosis: implications for the treatment of systemic sclerosis. J Invest Dermatol. Nov 1999;113(5):843-7. [Medline].

  80. Yung A, Reay N, Goodfield MD. Improvement in digital flexibility and dexterity following ingestion of sildenafil citrate (viagra) in limited systemic sclerosis. Arch Dermatol. Jul 2005;141(7):831-3. [Medline].

  81. Zakrzewska-Pniewska B, Jablonska S, Kowalska-Oledzka E, Blaszczyk M, Hausmanowa-Petrusewicz I. Sympathetic skin response in scleroderma, scleroderma overlap syndromes and inflammatory myopathies. Clin Rheumatol. 1999;18(6):473-80. [Medline].

 
Previous
Next
 
Face of 65-year old woman with systemic sclerosis and skin thickening of 20 years' duration: Note the pinched nose, taut skin with numerous telangiectasias, and retraction of the lips.
Telangiectasias affecting the face: They are pronounced and numerous, especially in the atrophic phase of the disease. Radical furrowing around the mouth is also characteristic in the later stage of the disease.
Raynaud phenomenon of the hands: Symmetrical acral vasospasm is present, with characteristic pallor, cyanosis, suffusion, and a sense of fullness and tautness.
Puffy appearance of the woman's hand in the edematous phase of early scleroderma.
In systemic sclerosis, ulceration at the tip of the finger is regarded to be secondary to ischemia.
Hand of a woman with scleroderma of several years' duration: The thickened, tight, thin skin over the fingers is the result of self-amputation of the distal phalanx due to ischemia. Moderately severe flexion contractures of the fingers are present.
In systemic sclerosis, skin hyperpigmentation of the lower legs is surrounded by areas of hypopigmentation. The result is a salt-and-pepper appearance.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.