Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Medication

  • Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 16, 2015
 

Medication Summary

Glucocorticoids can be used systemically, topically in ointment form, or under occlusive dressings. Diluted triamcinolone acetonide suspension of 1:3 to 1:5 given by intralesional injection has been suggested. Repeated infiltrations are recommended every 3-4 weeks. Ointments containing heparin or heparinoids may also be helpful.

Linear scleroderma in children has effectively been treated with oral calcitriol. The use of phenytoin has been advocated, especially for linear morphea. Long-term treatment with oral p-aminobenzoate has been suggested, but the results are unclear.

Topical tocoretinate for 6 months to 3 years may be beneficial for treating skin sclerosis.[4] The following agents have been reported to be beneficial in morphea: d-penicillamine, corticosteroids, phenytoin, aminobenzoate potassium, dimethyl sulfoxide, vitamin E, disodium edetate, sulfasalazine, cyclofenil, methotrexate, colchicine, antimalarials, azathioprine, griseofulvin, penicillin, chlorambucil, cyclophosphamide, etretinate, isotretinoin, and interferon alfa and gamma.

No treatment seems to alter the natural course of localized scleroderma, but reports indicate that moderate doses of systemic or locally injected corticosteroids, d-penicillamine, and photochemotherapy with 8-methoxypsolaren have been helpful in decreasing the degree of inflammation and fibrosis.

Next

Antimalarials

Class Summary

These agents have anti-inflammatory properties and inhibit multiple functions of phagocytes, including reactive oxygen species release.

Hydroxychloroquine (Plaquenil)

 

Treats malaria, rheumatoid arthritis, systemic lupus erythematosus, and juvenile chronic arthritis. Use in localized fibrosing disorders has not been well characterized, but several reports indicate some efficacy. Response to antimalarials is slow, so it can take up to 6 months for full effect.

Previous
Next

Corticosteroids

Class Summary

These agents have potent anti-inflammatory and immunosuppressive properties. They inhibit lymphocyte proliferation and delayed-type hypersensitivity and cause changes in WBC traffic and Fc receptor suppression.

Prednisone (Orasone, Deltasone, Sterapred)

 

Constitutes main therapy for multiple inflammatory and autoimmune disorders including vasculitis, systemic lupus erythematosus, inflammatory myopathies, and polymyalgia rheumatica. May be useful in localized and regional fibrosing disorders by inhibiting inflammatory response.

Triamcinolone (Aristocort)

 

Can be helpful in inhibiting inflammation and fibrosis in localized fibrosing conditions. Repeated administrations might be necessary.

Previous
Next

Immunosuppressives/disease-modifying antirheumatic drugs

Class Summary

These agents have anti-inflammatory and immunosuppressive properties. Some drugs may have antifibrotic effects.

D-Penicillamine (Cuprimine)

 

Used to treat rheumatoid arthritis and shown to have some benefit in systemic sclerosis. Its mode of action is unknown, but seems to modulate the immune system via sulfhydryl exchange reactions in various cells.

Azathioprine (Imuran)

 

Purine analog and derivative of 6-mercaptopurine. Has immunosuppressive effects by inhibiting purine synthesis in cells. Used for treating systemic lupus erythematosus, rheumatoid arthritis, vasculitis, and inflammatory myopathies and preventing allograft rejection.

Sulfasalazine (Azulfidine)

 

Sulfonamide derivative with anti-inflammatory properties. Useful for treating rheumatoid arthritis, spondyloarthropathies, and inflammatory bowel disease. Effects on localized fibrosing conditions are not well characterized.

Methotrexate (Rheumatrex)

 

This drug reversibly inhibits dihydrofolate reductase; limits the availability of 1-carbon fragments necessary for synthesis of purines and the conversion of deoxyuridylate to thymidylate in the synthesis of DNA and cell reproduction. Extensively used for cancer treatment, rheumatoid arthritis, psoriasis, and as a steroid-sparing agent in various autoimmune conditions.

Cyclophosphamide (Cytoxan, Neosar)

 

Synthetic nitrogen mustard alkylating agent used for treating severe lupus complications, vasculitis, refractory rheumatoid arthritis, scleroderma lung disease, and myopathies. Its role in localized forms of fibrosis has not been well characterized. It is indicated only for severe inflammatory lesions that do not respond to other agents.

Colchicine

 

Inhibits microtubule formation in leukocytes and decreases joint inflammation. Drug has no direct analgesic properties. Useful in gout, skin vasculitis, and Behçet disease. Has been advocated to have an antifibrotic effect with potential therapeutic implications in generalized or localized fibrosing disorders.

Tacrolimus (Prograft, Protopic)

 

Used to treat atopic dermatitis and for prophylaxis of renal and kidney transplant rejection.

Previous
Next

Topical Skin Product

Class Summary

Agents such as imiquimod may induce mediators of immune processes.

Imiquimod (Aldara)

 

Topical antiviral agent. Induces secretion of interferon alpha and other cytokines; mechanism of action is unknown.

Previous
 
 
Contributor Information and Disclosures
Author

Mariana J Kaplan, MD Assistant Professor, Department of Internal Medicine, Division of Rheumatology, University of Michigan Medical School

Mariana J Kaplan, MD is a member of the following medical societies: American Association of Immunologists, American Federation for Medical Research, Central Society for Clinical and Translational Research, Clinical Immunology Society, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

References
  1. Todd DJ, Askari A, Ektaish E. PUVA therapy for disabling pansclerotic morphoea of children. Br J Dermatol. 1998 Jan. 138(1):201-2. [Medline].

  2. Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. 2005 Jan. 152(1):180-2. [Medline].

  3. Namazi MR. Imiquimod: a potential weapon against morphea and fibromatoses. J Drugs Dermatol. 2004 Jul-Aug. 3(4):362-3. [Medline].

  4. Mizutani H, Yoshida T, Nouchi N, et al. Topical tocoretinate improved hypertrophic scar, skin sclerosis in systemic sclerosis and morphea. J Dermatol. 1999 Jan. 26(1):11-7. [Medline].

  5. Dehen L, Roujeau JC, Cosnes A, et al. Internal involvement in localized scleroderma. Medicine (Baltimore). 1994 Sep. 73(5):241-5. [Medline].

  6. Eguchi T, Harii K, Sugawara Y. Repair of a large "coup de sabre" with soft-tissue expansion and artificial bone graft. Ann Plast Surg. 1999 Feb. 42(2):207-10. [Medline].

  7. Falanga V, Medsger TA Jr, Reichlin M, et al. Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med. 1986 Jun. 104(6):849-57. [Medline].

  8. Ghersetich I, Teofoli P, Benci M, et al. Localized scleroderma. Clin Dermatol. 1994 Apr-Jun. 12(2):237-42. [Medline].

  9. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true connective tissue disease. Rheum Dis Clin North Am. 1996 Feb. 22(1):23-38. [Medline].

  10. Jablonska S, Blaszczyk M. Sclerodermalike diseases. Clin Dermatol. 1994 Jul-Sep. 12(3):437-48. [Medline].

  11. Kerscher M, Volkenandt M, Gruss C, et al. Low-dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. 1998 Jan. 38(1):21-6. [Medline].

  12. Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal fibrosis and histoplasmosis. Ann Thorac Surg. 1992 Dec. 54(6):1053-7; discussion 1057-8. [Medline].

  13. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995 Nov. 70(11):1068-76. [Medline].

  14. Schachter RK. Localized scleroderma. Curr Opin Rheumatol. 1989 Dec. 1(4):505-11. [Medline].

  15. Schumacher HR. Multifocal fibrosclerosis. Cecil Textbook of Medicine. WB Saunders Co; 2000. 1561-62.

  16. Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. 1997 Nov. 9(6):562-70. [Medline].

 
Previous
Next
 
This photograph shows morphea en plaque on the trunk of a patient. There is a distinctive border separating the plaque from the surrounding normal skin (reproduced with permission of Mayo Clinic Proceedings).
This photograph shows generalized morphea on the trunk of a patient (reproduced with permission from Mayo Clinic Proceedings).
CT scan of the abdomen showing the typical paraaortic mass of retroperitoneal fibrosis.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.