Quiz: April 2006


What is your diagnosis for this linear atrophic pigmented plaque?

What do you suggest as therapy? 




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Diagnosis: En coup de sabre

The most distinctive form of localized scleroderma is "en coup de sabre". Below, you can read the opinions of the iranderma members about the therapy of this disease;

 Dr. Reza Ghaderi; Associate Professor of Dermatology, Birjand UMS:

     1) Medical Care:  

  • 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.
  • 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 also can be useful.
  • Occasionally, other disease-modifying agents, including d-penicillamine, azathioprine, sulfasalazine, methotrexate, and cyclophosphamide, may be 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 usually are irradiated with 20 J/cm2 UVA1 for 12 weeks, with a cumulative UVA1 dose of 600 J/cm2.
  • A recent study reported that occlusive treatment with tacrolimus ointment can be useful in localized scleroderma. Imiquimod has also been suggested as a potential treatment for morphea and fibromatoses, but more studies are needed.

     2) Surgical Care:

  • Tendon-lengthening procedures and surgical release of joint contractures sometimes are 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, rarely may be necessary. Palmar fasciotomy is a useful and more benign procedure. Surgical management is often required for both retroperitoneal and mediastinal fibrosis to treat the complications.

    3) Consultations:

    • 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.

    4) Activity:

    • Physical therapy is very important for patients prone to develop joint and muscle contractures and deformities. Joint mobility should be maintained

      Dr. Hadi Nick, Romania:


         1.serologic test for Borrelia B. => + => tx. For B.B.

         2.topical tacrolimus

         3.Corticosteroids, penicillamine, methotrexate, calcipotriene, calcitriol, cyclosporine, and UVA-1 with modest results.

         4. Atrophic lesions also may be treated surgically with collagen injections and autologous fat transplantation.  

      Dr. Shahriar Nazari, Turkey:

       I think there is no effective treatment for this localized form of morphea and the only important point is to examine the involvement of deeper tissues. The disease is often self-limited; but if it is advancing, topical and/or intralesional steroids with or without UVA may be tried for example in this patient. So many medications have been tried in this disease, but the results are unclear (calcitriol, Vit.E, phenytoin, MTX, UVA therapy etc).  

      Dr. Akaberi, Iran, Yazd:

      Therapy is plastic surgery  

       Dr.  Nabil Al-Rabeei, Iraq:

      Regarding the treatment of localized scleroderma(morphea) remains unsatisfactory, but because the disease is

      generally self-limited, most cases require little or no treatment. when treatment is attempted is usually involves application of high-to

      ultrahigh-potency topical glucocorticoides to the lesions, which may be augmented by intra lesional injections of glucocorticoids.  

      Dr. Yalda Nahidi, Iran:

      Facial hemiatrophy is usually persistent and the only treatment for facial hemiatrophy is reconstructive surgery, but frontoparietal scleroderma may clear spontaneously, but if therapy is required we can use topical (intralesional steroids)[ alone or in combination with PUVA] , calcipotriol, tigasone and antimalarial agents. systemic corticosteroids can be used in it's inflammatory stage.  

      Dr. Mehrdad Mehravaran, Hungary:

      • Calcipotriene (Daivonex®) ointment 0.005% applied twice daily with occlusion.
      • In rapidly progressive disease, systemic steroids up to 1 mg/kg per day, plus methotrexate 10–25 mg per week may be effective in arresting progression of the condition.

      ·        Cyclosporine 3 mg/kg/day for a trial of 1–2 months may be considered in patients failing the above treatments.

      ·        In patients treated with systemic steroids and methotrexate, once the condition is controlled, the agents are gradually tapered over months to several years.

      ·        After the linear morphea is controlled and burned out, cosmetic procedures are indicated to replace lost dermis and subcutaneous tissue. Fillers and fat transplantation, or excision of affected areas with tissue expansion, if required, may be considered. Surgery does not appear to "reactivate" linear scleroderma.

      Should take care:

      ·        Linear scleroderma can result in significant permanent sequelae. Therapy should especially be considered in younger children that might produce permanent disfigurement. In older children and adults therapy may be less aggressive as the complications may be less severe. Consider the risk versus the possible benefit before undertaking immunosuppressive therapy.

      ·        Patients with limited cutaneous disease may have involvement of underlying tissues such as bone, brain, and lung. Appropriate evaluations are indicated if symptoms suggest involvement of deeper tissues.  

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