Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Treatment & Management
- Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD more...
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- Most patients with plaque morphea experience spontaneous remission and require no specific treatment. Treatment depends entirely on the severity of the findings. Intralesional injections of corticosteroids might be helpful in early stages.
- The progression of Dupuytren contracture varies, ranging from little or no change over many years to rapid progression and complete flexion contracture of one or more digits.
- The treatment of mediastinal and retroperitoneal fibrosis has not been well studied, although corticosteroids, immunosuppressive agents, and tamoxifen appear to be effective.
- If the lesions spread (as in generalized morphea), anti-inflammatory or immunosuppressive medications may be indicated.
- Although numerous therapeutic agents have been used for morphea, treatment remains unsatisfactory.
- Daily antimalarial agents may be beneficial, especially when lesions are highly inflammatory.
- In linear scleroderma and deep morphea, aggressive treatment, including systemic corticosteroids, may be necessary. Topical corticosteroids may also be useful.
- Occasionally, other disease-modifying agents, including d-penicillamine, azathioprine, sulfasalazine, methotrexate, and cyclophosphamide, are necessary to control a severe inflammatory process.
- Plasmapheresis may be useful in some patients, but no randomized controlled trials have been published.
- Reports indicate that patients with severe localized scleroderma have been treated successfully with psoralen plus ultraviolet light of the A wave length (PUVA) bath photochemotherapy.
- Low-dose UVA1 phototherapy can be highly effective for sclerotic plaques, even in patients with advanced localized fibrosing disorders with rapidly evolving lesions despite conventional therapy.
- Patients are usually irradiated with 20 J/cm2 UVA1 for 12 weeks, with a cumulative UVA1 dose of 600 J/cm2.
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- Tendon-lengthening procedures and surgical release of joint contractures are sometimes necessary.
- Amputation may be necessary as a consequence of severe flexural deformity.
- Often, patients with en coup de sabre or Parry-Romberg syndrome require surgical reconstruction of the face and scalp.
- Reports indicate that en coup de sabre lesions have been repaired effectively with a combination of an expanded skin flap and a hydroxyapatite implant.
- When actual contractures occur in Dupuytren contracture, surgical intervention is desirable. Limited fasciotomy is effective in most instances. More radical procedures, including amputation, are necessary in rare cases. Palmar fasciotomy is a useful and more benign procedure. Surgical management is often required to treat the complications of both retroperitoneal and mediastinal fibrosis.
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- Patients with the linear and deep types of morphea require physical therapy to prevent joint deformities and skin contractures. Heat treatment and massage might be helpful.
- Psychotherapy for people with deformities and disfiguration is very important.
- Early evaluation by a reconstructive or plastic surgeon is important for patients with en coup de sabre lesions or Parry-Romberg disease.
- Evaluation by a hand surgeon may be indicated in patients with Dupuytren contracture for consideration of releasing the contractures.
- Surgical consultation may be considered in patients with mediastinal and retroperitoneal fibrosis if obstructive lesions occur.
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- Physical therapy is very important for patients prone to develop joint and muscle contractures and deformities. Joint mobility should be maintained.
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