| Localized Scleroderma: Morphea | |
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| Morphea Treatments |
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| Overview of Morphea Treatments |
| Many cases of typical plaque morphea entail only one or two “mild” morphea plaques which are not rapidly growing or spreading; are not on the face; are not over joints; and are only on the surface and not digging down deep into muscles and other tissues. Such cases typically do not require treatment, and will likely improve within two or three years, even without any treatment. |
| Localized scleroderma. Studies over the past year highlight the wide range of extracutaneous manifestations and different forms of localized scleroderma and suggest that treatment may be beneficial. PubMed. Curr Opin Rheumatol. 2006 Nov;18(6):606-13. |
| Rarer types of morphea, or those that are rapidly spreading, highly visible, over joints, or growing deep into underlying tissues may require prompt and more aggressive treatment to prevent the spread of plaques and to minimize damage from the disease, since the more severe cases of morphea may cause permanent disfigurement or disability. |
| Since all types of scleroderma are rare, most physicians may see only one or two cases in an entire career. Thus, it is very difficult for them to identify, categorize, and responsibly advise and treat morphea patients. |
| There are a few dozen centers in the world that specialize in scleroderma, and of those, only a few treat children. (See Scleroderma Experts) |
| Localized scleroderma (morphea). Treatment should be decided according to severity and extent of lesions. Limited lesions may be treated with local steroids such as class IV corticosteroids. Systemic treatment (methotrexate) should be discussed in extensive and linear forms when there is a risk of functional or esthetic complications. PubMed. Presse Med. 2006 Dec;35(12 Pt 2):1923-8. |
| Some of the treatments mentioned here include Apligraf, Calcipotriol cream, endermology, Imiquimod cream, laser, methotrexate and glucorti phototherapy (UVA, PUVA), and Tacrolimus cream. No treatment, medication or ointment has been proven by large-scale placebo-controlled studies to alter the course of morphea scleroderma. |
| A placebo-controlled study is still necessary to confirm the methotrexate and glucorticorticoid combination therapy; and, by any means, these are toxic therapies, particularly in children. |
| Scleroderma Experts |
| Adult morphea patients may be referred to any of the scleroderma expert centers. See the worldwide listing of the Scleroderma Clinical Trials Consortium (SCTC) for participating physicians and their contact information. |
| Juvenile scleroderma patients have fewer options, since many of the SCTC centers do not treat children or adolescents. Since the consequences of untreated morphea can be severe in some cases, it is important to seek expert advice as soon as possible. Refer to this listing: |
| ISN's Voices of Scleroderma Volume 1 book features juvenile scleroderma expert, Dr. Thomas Lehman, who wrote the medical overview of juvenile scleroderma. It also features chapters of true stories by juvenile scleroderma patients and caregivers. |
| Voices of Scleroderma Volume 2 features juvenile scleroderma expert Dr. L. Nandini Moorthy, who wrote the medical overview of juvenile scleroderma. It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. It is an excellent all-around book for patients and caregivers! |
| Voices of Scleroderma Volume 3 features juvenile scleroderma expert Dr. Fernanda Falcini of Italy, who wrote the medical overview of juvenile scleroderma. It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. It is another excellent all-around book for patients and caregivers! |
| Screening for Conditions Associated with Morphea |
| Conditions Associated with Morphea Scleroderma. About 25% of morphea patients will have at least one extra-cutaneous (non-skin) manifestation, but less than 4% of morphea patients will develop more than two non-skin manifestations. Therefore patients with morphea should receive an annual physical exam to monitor for any additional symptoms which may develop. They should also be tested for anticentromere antibodies (ACA), since the presence of ACA antibodies identifies the very small percentage of morphea patients who are at risk for progressing to systemic sclerosis. ISN. (See: Conditions Associated with Morphea Scleroderma) |
| Apligraf for Bullous Morphea Ulcers |
| Ulcers caused by bullous morphea treated with tissue-engineered skin. We report a patient with bullous morphea with long-standing ulcers whom we successfully treated with the tissue-engineered skin Apligraf (Organogenesis Inc., Canton, MA). The patient experienced rapid improvement in granulation tissue and the ulcers healed 4 months after a single application. PubMed. Int J Dermatol 2003 May;42(5):402-4. |
| Bosentan (Tracleer) |
| New developments in localized scleroderma. Previous results, using methotrexate and phototherapy, have been confirmed. A successful use of bosentan, an endothelin receptor antagonist with vasodilatative and antifibrotic properties for refractory cutaneous ulcerations in pansclerotic morphea, opens new horizons of treatment. Zulian, Francesco. Current Opinion in Rheumatology. 20(5):601-607, September 2008. |
| Endermology or LPG |
| Effectiveness of LPG(R) treatment in morphea. The LPG(R) technique, also known as Endermology(R) treatment, is a noninvasive technique consisting of a tissue mobilization process in which a skin fold is created between two rollers, stretching the underlying tissue and mobilizing the fold. It is an adjunctive treatment for morphea. It cannot eliminate the disease but can relieve the pain, soften the skin and improve the quality of life for these patients. PubMed. J Eur Acad Dermatol Venereol. 2004 Sep;18(5):527-30. |
| Imiquimod Cream |
| Use of Imiquimod Cream 5% in the Treatment of Localized Morphea. In this case report, imiquimod cream 5% (Aldara®), which induces interferon and in turn inhibits TGF-beta, was employed to treat morphea. PubMed. J Cutan Med Surg. 2004 May 3. |
| Laser |
| Laser Doppler flowmetry (LDF) for assessing localized scleroderma in children. LDF is a helpful, noninvasive diagnostic technique that can be used to discriminate disease activity in children with localized scleroderma, and is more accurate than thermography for this purpose. Weibel L. (PubMed) Arthritis Rheum. 2007 Oct;56(10):3489-95. |
| Alterations of basement membrane zone and cutaneous microvasculature in morphea and extragenital lichen sclerosus. Three-dimensional reconstruction of the skin vascular network showed increased angiogenesis only in the early inflammatory stage of morphea, whereas in inactive morphea and lichen sclerosus various numbers of enlarged vessels were visible. The changes in the vascular network in morphea appear to be related to the activity of the disease. Am J Dermatopathol. 2005 Dec;27(6):489-96. (Also see: Lichen Sclerosus) |
| Use of a 585 nm pulsed dye laser for the treatment of morphea. Pulsed dye laser therapy is a viable treatment option for morphea. Dermatol Surg. 2002 Jul;28(7):615-6. Entrez PubMed. |
| Methotrexate and Glucocorticoids |
| It is very wise to consult a scleroderma expert for guidance in determining which cases may require this treatment and to establish the treatment protocol. A placebo-controlled study is still necessary to confirm the methotrexate/ glucocorticoid therapy; and, by any means, these are toxic therapies, particularly in children. |
| Pediatric morphea (localized scleroderma): Review of 136 patients. These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. (Galenicom). Journal of the American Academy of Dermatology. June 19, 2008. |
| Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphea) in children. These data suggest that systemic corticosteroids and methotrexate in combination are beneficial and well tolerated in the treatment of children with LS. Long-term monitoring is mandatory. IngentaConnect. Br J Dermatol. 2006 Nov;155(5):1013-20. (Also see: Juvenile Scleroderma) |
| Treatment of Pediatric Localized Scleroderma with Methotrexate. Methotrexate appears to be a safe and effective therapy for pediatric Localized Scleroderma. S. J Rheumatol 2006; 33:609–14. (Also see: Juvenile Scleroderma) |
| Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed. PubMed. Arch Dermatol. 2005 Jul;141(7):847-52. (Also see: Linear Scleroderma) |
| Phototherapy, UVA, PUVA |
| Effect of Increased Pigmentation on the Antifibrotic Response of Human Skin to UV-A1 Phototherapy. Clinical responses of sclerotic skin to UV-A1 phototherapy were modest because of UV-A1–induced skin darkening, which is photoprotective and attenuates antifibrotic responses. Arch Dermatol. 2008;144(7):851-858. (Also see: Graft-vs-Host Disease) |
| Ultraviolet Light Therapy Is as Beneficial for Darker Skin as Lighter Skin. An analysis of more than 100 patients has confirmed for the first time that darker-skinned patients benefit as those with lighter skin when given light therapy for morphea and related diseases. Newswise. UT Southwestern Medical Center. 07/08/08. (Also see: Skin Involvement) |
| Efficacy of UVA1 phototherapy in 230 patients with various skin diseases. Besides topical and systemic therapy, UVA1 radiation is a good option of treatment in various skin diseases. It is one of the first-line treatments for several sclerotic diseases and it often improves pruritus considerably. (PubMed) Photodermatol Photoimmunol Photomed. 2008 Feb; 24(1):19-23. (Also see: Scleroderma Treatments) |
| Photodynamic therapy: other uses. The ability of this treatment to hone in on dysplastic epithelial and endothelial cells while retaining viability of surrounding tissue is its key feature because this leads to specific tumor destruction with cosmesis and function of the target organ intact. PubMed. Dermatol Clin. 2007 Jan;25(1):101-9. (Also see: Skin Fibrosis, and Clinical Trials: Positive Results: Phototherapy) |
| A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma (LS). Phototherapy, as previously reported in several noncontrolled trials, is an effective therapeutic option in LS, with a favorable risk/benefit ratio. UVA1 phototherapy should be considered among the first approaches in the management of LS. PubMed. J Am Acad Dermatol. 2006 Mar;54(3):440-7. (Also see: Scleroderma Clinical Trials: Positive Results) |
| Photodynamic therapy (PDT) in dermatology. A therapeutical benefit of PDT is also evident for inflammatory dermatoses like localized scleroderma, acne vulgaris and granuloma annulare. The benefits of PDT are the low level of invasiveness and the excellent cosmetic results after treatment. PubMed. Photodermatol Photoimmunol Photomed. 2005 Jun;21(3):142-9. (Also see: Linear Scleroderma) |
| Phototherapy and photochemotherapy of sclerosing skin diseases. Two phototherapeutic modalitites are used for the treatment of sclerosing skin diseases, long-wave ultraviolet A and psoralen plus ultraviolet A (PUVA). PubMed. Photodermatol Photoimmunol Photomed. 2005 Jun;21(3):157-65. (Also see: Linear Scleroderma) |
| Phototherapy for skin disease The sun can be one of our skin’s worst enemies, but now some dermatologists are taking clues from sunlight to battle a painful skin disease with a new type of phototherapy. MedicalNewsToday. 03/02/04. |
| Tacrolimus Cream |
| FDA Public Health Advisory. Elidel (pimecrolimus) Cream and Protopic (tacrolimus) Ointment. There is a potential cancer risk from use of Elidel (pimecrolimus) and Protopic (tacrolimus). It may take ten years or longer to determine if they are linked to cancer. Elidel and Protopic should be used only as labeled, for patients who have failed treatment with other therapies. FDA. 03/10/05. |
