Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Treatment & Management

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. Surgical treatment is often highly successful. Steroid therapy may be useful in the cases detected before significant obstruction has occurred. Azathioprine, cyclophosphamide, and tamoxifen have been used as well, with apparent success. The long-term outlook is fairly good if the disease is recognized early before irreversible obstructive lesions have occurred. Most deaths are secondary to renal failure.

Threapy for specific disorders is as follows:

• Progressive hemifacial atrophy (PHA) – Methotrexate (MTX) is the standard therapy for active  PHA. A long course of therapy is typically required, as relapse is frequently seen with shorter courses of therapy. Current evidence supports a 12-24 month course of methotrexate being most effective in inducing prolonged remission. MTX is often combined with oral prednisone over the first three months because MTX has a delayed effect on inflammation and fibrosis. ^[4]  If the lesions spread (as in generalized morphea), anti-inflammatory or immunosuppressive medications may be indicated.
• Morphea – First-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. Mycophenolate mofetil or mycophenolic acid, used alone or in combination with other systemic therapies has been reported to improve or stablize disease in  severe morphea. ^[6]  Daily antimalarial agents may be beneficial, especially when lesions are highly inflammatory. ^[7]
• 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.

One study reported that occlusive treatment with tacrolimus ointment can be useful in localized scleroderma. ^[8]  Imiquimod has been suggested as a potential topical treatment for morphea and fibromatoses, but more studies are needed. ^[9]

Corticosteroids are considered first-line therapy for eosinophilic fasciitis (ET), however prolonged use is often required. In one study, complete response was more likely with a combination of corticosteroids and methotrexate (64%) than corticosteroid monotherapy (30%). ^[5]  

Successful treatment of morphea with abatacerpt, a recombinant fusion protein interfering with the T-cell costimulatory pathway, has been reported in adults as well as in a small case series of pediatric patients. ^[10]

Phototherapy

Several phototherapy modalities including ultraviolet B (UVB), psoralen plus broadband UVA (PUVA), broadband UVA, and UVA1 have been investigated for morphea. Lower wave length phototherapy (UVA) has emerged as the preferred modality because of its greater potency of tissue penetration than UVB and lower risk of sunburn than broadband UVA. Evidence suggests that UVA1 effects are dose-related and superior results for high-dose-UVA compared with low-dose-UVA have been reported. ^[1]   

There are no internationally agreed definitions of treatment doses but in general, categories are as follows ^[11] :

• Very low dose: < 10 J/cm ² i
• Low dose: 10-29 J/cm ² 
• Medium dose: 30-59 J/cm ² 
• High dose: 60-130 J/cm ² 

Long-term outcome of UVA1 therapy is unclear. In a study of 37 adults who underwent successful UVA1 therapy, 2-year and 3-year recurrence rates of 44.5 and 48.4%, respectively. Active morphea for more than 1 year prior to UVA treatment significantly increased the risk of recurrence. ^[12]

Acute adverse effects of UVA1 are minimal, with mild tanning or skin pigmentation being the most common. Uncommon acute adverse effects include reactivation of herpes simplex, cholinergic urticaria, and transient and reversible changes in the appearance of moles The risk of long-term adverse effects, particularly skin cancer, is unknown. ^[11]

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.

Consultations to consider include the following:

• Physical therapy is very important for patients prone to develop joint and muscle contractures and deformities. Joint mobility should be maintained. Heat treatment and massage might be helpful.
• Psychotherapy for people with deformities and disfiguration is very important.
• Cases that involve children with severe facial deformities should be referred to a plastic surgeon for prompt evaluation and consultation about possible treatment.
• 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.

Periodic follow-up is recommended to detect early spread of lesions or other chronic complications.