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Dermatologic Manifestations of Mixed Connective Tissue Disease Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 13, 2016
 

Medication Summary

Mixed connective-tissue disease (MCTD) is a chronic and usually mild disease, which can be treated symptomatically or with corticosteroids or immunosuppressives if the severity of disease justifies it. Combined treatment allows dose reduction of systemic steroids (corticosteroid-sparing effect). Treatment depends on internal organ involvement. Midrange doses of systemic corticosteroids have been used in conjunction with immunosuppressive agents. Anti-inflammatory agents are helpful for arthralgia, myalgia, and swelling of the hands. Skin lesions can be treated with topical corticosteroids. In all cases, photoprotection is recommended.

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Corticosteroids, systemic

Class Summary

These agents decrease autoimmune reactions, possibly by suppressing key components of the immune system. They are often used to treat collagen vascular diseases.

Prednisolone (Prelone Syrup, Pediapred Oral Solution, Delta-Cortef)

 

Prednisolone is a synthetic adrenocortical steroid with predominantly glucocorticoid properties. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reducing capillary permeability.

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Immunosuppressants

Class Summary

These agents inhibit key factors that mediate immune reactions, which in turn decrease inflammatory responses.

Cyclosporine (Sandimmune, Gengraf, Neoral)

 

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease) in a variety of organs. For children and adults, base dosing on ideal body weight. Onset of action is 1-2 months; stabilization of disease may take 3-4 months. Bioavailability is not necessarily equal in different formulations; use caution. In severe disease, higher doses (transplant dosing) increases risk of adverse events and may be beyond the scope of practice for a general dermatologist.

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Nonsteroidal anti-inflammatory drugs

Class Summary

NSAIDs provide symptomatic relief for arthralgia, myalgia, edema, and tenderness.

Naproxen (Naprosyn, Anaprox)

 

Naproxen is used for relief of mild to moderate pain; it inhibits inflammatory reactions and pain by decreasing the activity of cyclo-oxygenase, which decreases prostaglandin synthesis.

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Corticosteroids, topical

Class Summary

Topical corticosteroids are moderate or potent agents with anti-inflammatory and immunosuppressive properties. They can decrease epidermal proliferation.

Fluticasone (Cutivate)

 

Fluticasone has extremely potent vasoconstrictive and anti-inflammatory activities. It has a weak inhibitory affect on the hypothalamic-pituitary-adrenocortical axis when applied topically. Other more potent topical corticosteroids may be useful for unresponsive inflammatory skin lesions. Less potent nonfluorinated topical corticosteroids may be useful in patients with less aggressive skin disease. Fluticasone is of medium potency.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

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

Disclosure: Nothing to disclose.

Coauthor(s)

Anna Wozniacka, MD, PhD Professor, Department of Dermatology, Medical University of Lodz, Poland

Anna Wozniacka, MD, PhD is a member of the following medical societies: European Academy of Dermatology and Venereology, Polish Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Federation for Medical Research, American Society for Clinical Investigation, Society for Investigative Dermatology

Disclosure: Received consulting fee from Novan for consulting; Received consulting fee from Stieffel, a GSK company for consulting; Received salary from Society for Investigative Dermatology for board membership.

Acknowledgements

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

Disclosure: Nothing to disclose.

References
  1. Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, et al. The diagnosis and classification of mixed connective tissue disease. J Autoimmun. 2014 Feb-Mar. 48-49:46-9. [Medline].

  2. Aringer M, Steiner G, Smolen JS. Does mixed connective tissue disease exist? Yes. Rheum Dis Clin North Am. 2005 Aug. 31(3):411-20, v. [Medline].

  3. Cappelli S, Bellando Randone S, Martinovic D, Tamas MM, Pasalic K, Allanore Y, et al. "To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity. Semin Arthritis Rheum. 2012 Feb. 41(4):589-98. [Medline].

  4. Yoshida K, Inoue H, Komai K, Yamane T, Hashiramoto A, Shiozawa K, et al. Mixed connective tissue disease is distinct from systemic lupus erythematosus: study of major histocompatibility complex class I polypeptide-related sequence A and HLA gene polymorphisms. Tissue Antigens. 2013 Jan. 81(1):44-5. [Medline].

  5. Kattah NH, Kattah MG, Utz PJ. The U1-snRNP complex: structural properties relating to autoimmune pathogenesis in rheumatic diseases. Immunol Rev. 2010 Jan. 233(1):126-45. [Medline].

  6. Paradowska-Gorycka A, Stypińska B, Olesińska M, Felis-Giemza A, Mańczak M, Czuszynska Z, et al. Association of HLA-DRB1 alleles with susceptibility to mixed connective tissue disease in Polish patients. HLA. 2016 Jan. 87 (1):13-8. [Medline].

  7. Gunnarsson R, Molberg O, Gilboe IM, Gran JT. The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients. Ann Rheum Dis. 2011 Mar 11. [Medline].

  8. Ungprasert P, Crowson CS, Chowdhary VR, Ernste FC, Moder KG, Matteson EL. Epidemiology of Mixed Connective Tissue Disease 1985-2014: A Population Based Study. Arthritis Care Res (Hoboken). 2016 Mar 4. [Medline].

  9. Ortega-Hernandez OD, Shoenfeld Y. Mixed connective tissue disease: An overview of clinical manifestations, diagnosis and treatment. Best Pract Res Clin Rheumatol. 2012 Feb. 26(1):61-72. [Medline].

  10. Hajas A, Szodoray P, Nakken B, Gaal J, Zöld E, Laczik R, et al. Clinical Course, Prognosis, and Causes of Death in Mixed Connective Tissue Disease. J Rheumatol. 2013 Jul. 40(7):1134-1142. [Medline].

  11. Grader-Beck T, Wigley FM. Raynaud's phenomenon in mixed connective tissue disease. Rheum Dis Clin North Am. 2005 Aug. 31(3):465-81, vi. [Medline].

  12. Hall S, Hanrahan P. Muscle involvement in mixed connective tissue disease. Rheum Dis Clin North Am. 2005 Aug. 31(3):509-17, vii. [Medline].

  13. Swart JF, Wulffraat NM. Diagnostic workup for mixed connective tissue disease in childhood. Isr Med Assoc J. 2008 Aug-Sep. 10(8-9):650-2. [Medline].

  14. Berard RA, Laxer RM. Pediatric Mixed Connective Tissue Disease. Curr Rheumatol Rep. 2016 May. 18 (5):28. [Medline].

  15. Oh YB, Jun JB, Kim CK, et al. Mixed connective tissue disease associated with skin defects of livedoid vasculitis. Clin Rheumatol. 2000. 19(5):381-4. [Medline].

  16. Yamamura K, Takahara M, Masunaga K, Sawabe T, Kitano M, Mashino T, et al. Subcutaneous calcification of the lower legs in a patient with mixed connective tissue disease. J Dermatol. 2011 Mar 21. [Medline].

  17. Usuba FS, Lopes JB, Fuller R, Yamamoto JH, Alves MR, Pasoto SG, et al. Sjögren's syndrome: An underdiagnosed condition in mixed connective tissue disease. Clinics (Sao Paulo). 2014 Mar. 69(3):158-62. [Medline]. [Full Text].

  18. Sen S, Sinhamahapatra P, Choudhury S, Gangopadhyay A, Bala S, Sircar G, et al. Cutaneous manifestations of mixed connective tissue disease: study from a tertiary care hospital in eastern India. Indian J Dermatol. 2014 Jan. 59(1):35-40. [Medline]. [Full Text].

  19. Weber P, Ganser G, Frosch M, Roth J, Hülskamp G, Zimmer KP. Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol. 2000 Nov. 27(11):2692-5. [Medline].

  20. Fagundes MN, Caleiro MT, Navarro-Rodriguez T, et al. Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med. 2009 Jun. 103(6):854-60. [Medline].

  21. Aalokken TM, Lilleby V, Soyseth V, et al. Chest abnormalities in juvenile-onset mixed connective tissue disease: assessment with high-resolution computed tomography and pulmonary function tests. Acta Radiol. 2009 May. 50(4):430-6. [Medline].

  22. Colin G, Nunes H, Hatron PY, Cadranel J, Tillie I, Wallaert B. [Clinical study of interstitial lung disease in mixed connective tissue disease]. Rev Mal Respir. 2010 Mar. 27(3):238-46. [Medline].

  23. Nowicka-Sauer K, Czuszynska Z, Majkowicz M, Smolenska Z, Jarmoszewicz K, Olesinska M, et al. Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus. Lupus. 2012 Mar 20. [Medline].

  24. Lundberg IE. Cardiac involvement in autoimmune myositis and mixed connective tissue disease. Lupus. 2005. 14(9):708-12. [Medline].

  25. Gourley I, Bell AL, Biggart D. Kikuchi's disease as a presenting feature of mixed connective tissue disease. Clin Rheumatol. 1995 Jan. 14(1):104-7. [Medline].

  26. Latuskiewicz-Potemska J, Zygmunt A, Biernacka-Zielinska M, Stanczyk J, Smolewska E. Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl. Postepy Dermatol Alergol. 2013 Oct. 30(5):329-36. [Medline]. [Full Text].

  27. Hoffmann-Vold AM, Gunnarsson R, Garen T, Midtvedt Ø, Molberg Ø. Performance of the 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis (SSc) in large, well-defined cohorts of SSc and mixed connective tissue disease. J Rheumatol. 2015 Jan. 42(1):60-3. [Medline].

  28. Rai R, Swetha T. Association of anti-phospholipid antibodies with connective tissue diseases. Indian Dermatol Online J. 2015 Mar-Apr. 6(2):89-91. [Medline]. [Full Text].

  29. Peller JS, Gabor GT, Porter JM, Bennett RM. Angiographic findings in mixed connective tissue disease. Correlation with fingernail capillary photomicroscopy and digital photoplethysmography findings. Arthritis Rheum. 1985 Jul. 28(7):768-74. [Medline].

  30. Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. 1972 Feb. 52(2):148-59. [Medline].

  31. Hof D, Raats JM, Pruijn GJ. Apoptotic modifications affect the autoreactivity of the U1 snRNP autoantigen. Autoimmun Rev. 2005 Jul. 4(6):380-8. [Medline].

  32. Hasegawa EM, Caleiro MT, Fuller R, Carvalho JF. The frequency of anti-beta2-glycoprotein I antibodies is low and these antibodies are associated with pulmonary hypertension in mixed connective tissue disease. Lupus. 2009 Jun. 18(7):618-21. [Medline].

  33. Seguchi M, Soejima Y, Tateishi A, et al. Mixed connective tissue disease with multiple organ damage: successful treatment with plasmapheresis. Intern Med. 2000 Dec. 39(12):1119-22. [Medline].

  34. Lage R, Biccigo DG, Santos FB, Chimara E, Pereira ES, Costa Ad. Mycobacterium chelonae cutaneous infection in a patient with mixed connective tissue disease. An Bras Dermatol. 2015 Jan-Feb. 90(1):104-7. [Medline]. [Full Text].

  35. Guo RF, Gebreab FH, Tang EH, Piao Z, Lee SS, Perez ML. Cutaneous ulcer as leading symptom of systemic cytomegalovirus infection. Case Rep Infect Dis. 2015. 2015:723962. [Medline]. [Full Text].

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Sclerodermalike changes on the face.
Sausage-shaped fingers in a patient with Raynaud phenomenon.
Direct immunofluorescence testing shows epidermal nuclear immunoglobulin G staining.
Indirect immunofluorescence testing shows the typical speckled pattern for ribonucleoprotein antibodies on a HEp-2 substrate.
 
 
 
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