Apligraf for Bullous Ulcers
Endermology or LPG
Methotrexate and Glucocorticoids
Phototherapy, UVA, PUVA
Tacrolimus Cream: Warning
UVA1 phototherapy is now the recommended first-line treatment for morphea.
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.
Ultraviolet A1 phototherapy beyond morphea: experience in 83 patients. UVA1 phototherapy is effective and safe in the treatment of graft–versus–host disease, nephrogenic systemic fibrosis, systemic sclerosis, and mast cell disorders. PubMed, Photodermatol Photoimmunol Photomed, 2015 Nov;31(6):289-95. (Also see Skin Fibrosis and Graft-versus-Host Disease)
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)
See the ISN Scleroderma Experts section, where each center is identified as to whether or not they treat adults and/or juveniles, and whether or not they treat localized forms of scleroderma, such as morphea and linear.
Very few of the expert centers treat children with localized scleroderma. Since the consequences of untreated morphea can be severe in some cases, it is important to seek expert advice as soon as possible.
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 1 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 3features 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!
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.
Cancer: T-Cell Lymphoma
Neonatal Soft Tissue Reconstruction Using a Bioengineered Skin Substitute. This was serially debrided and ultimately repaired using Apligraf (Graftskin, Living Skin Equivalent, LSE; Organogenesis Inc, Canton, MA), which rapidly facilitated secondary healing. PubMed, J Craniofac Surg, 2017 Mar;28(2):489-491.
Localized scleroderma and zinc: a pilot study. We conclude that high-dose zinc gluconate can therefore be a valuable alternative treatment for localized scleroderma, with good tolerability, although placebo-controlled studies are necessary to confirm our results. PubMed, Brocard A, Eur J Dermatol. (Also see Alternative Therapies)
Camouflage makeup, or stage and screen makeup may be effective for temporary coverage of morphea patches, on the face or body. This type of makeup can hold up under tough conditions like swimming. When properly applied, it won't wear off on clothing, and a single application can last for days.
Dermacolor Camouflage by Kryolan makeup is an example of longlasting professional makeup, as used by actors and models. When properly applied, it can provide suitable coverage for skin colorations such as telangiectasia, birth marks, and morphea scleroderma. (Also see Telangiectasia, Corrective Cosmetics)
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.
Case Report: 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.
Non–invasive Imaging of Localised Scleroderma for Assessment of Skin Blood Flow and Structure. This novel study of optical coherence tomography, high–frequency ultrasound, laser Doppler imaging and thermography confirms loss of epidermal thickness and increased deeper perfusion in morphea plaques. PubMed, Acta Derm Venereol, 2016 Jun 15;96(5):641-4.
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.
Long-Term Methotrexate Efficacy in Juvenile Localized Scleroderma. Methotrexate (MTX) shows a prolonged efficacy in patients with juvenile localized scleroderma (JLS), with partial or complete remissions sustained in most of the patients, even after two years. Pediatric Rheumatology.
For UVA1 treatment, first contact your doctor to see if it they will recommend it for you. It is not used for lichen sclerosus that is on the genital area. If they recommend and prescribe it for you, try to find UVA1 facilities near you. Some dermatology offices (usually large offices or with major health centers) have UVA1 equipment (not UVB). It may also be possible to buy UVA1 phototherapy equipment for home use. It is manufactured by Daavlin and distributed worldwide. Home users must be monitored by a physician, who will prescribe the necessary dosage and monitor for possible side-effects, such as skin cancer.
Comparing ultraviolet light A photo(chemo)therapy with Methotrexate (MTX) protocol in childhood localized scleroderma: Evidence from systematic review and meta–analysis approach. Our study supports the combination of MTX and glucocorticoids in patients with a high risk of complication and UVA1 could represent a therapeutic option in patients with limited scleroderma. Seminars in Arthritis and Rheumatism, 03/30/2018. (Also see Immunosuppressants)
Case Report: Improvement of peripheral artery disease with Sildenafil and Bosentan combined therapy in a patient with limited cutaneous systemic sclerosis. Sildenafil and Bosentan combined therapy was associated in our case with an improvement of the maximum walking distance without adverse effect. PMC, Medicine (Baltimore), 2017 Jun; 96(25): e6988.
The dark side of the light: Phototherapy adverse effects. Despite concerns over the carcinogenic potential of ultraviolet radiation, most studies have not found an increased risk of non–melanoma or melanoma skin cancer in patients treated with ultraviolet B (broadband and narrowband) and ultraviolet A1 phototherapy. PubMed, Clin Dermatol, 2016 Sep-Oct;34(5):556-62. (Also see Cancer and Clinical Trials: Positive Results)
Phototherapy in Scleroderma. More rigorous studies are needed to evaluate phototherapy in the treatment of scleroderma, however, based on the limited studies available, 20-50 J/cm2 of UVA1 therapy 3–4 times a week for 30 treatments is recommended. PubMed, Dermatol Ther (Heidelb), 08/12/2016.
Low-dose UVA1 phototherapy for scleroderma: what benefit can we expect? In patients with morphea, a marked improvement was found in 77.8% patients and a moderate improvement was found in 11.1% patients. In the systemic scleroderma group, a patient with complete remission of the skin sclerosis is emphasized. PubMed, J Eur Acad Dermatol Venereol. (Also see Skin Fibrosis Treatments)
Ultraviolet A1 phototherapy: a British Photodermatology Group workshop report. UVA1 is currently underprovided, and the recommendation of the workshop is that more tertiary centres should have access to UVA1 phototherapy in the UK. PubMed.
(Expired Article) 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. Medscape.
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