Materiały źródłowe

• #1 Morphea (localized scleroderma) in adults: Management – UpToDate

  https://www.uptodate.com/contents/morphea-localized-scleroderma-in-adults-management

  Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Affected patients present with single or multiple inflammatory and sclerotic plaques, findings considered manifestations of active disease. These plaques eventually become inactive, leaving permanent dermal or soft tissue atrophy and pigmentary changes (skin damage). […] There are multiple treatment options for active morphea; however, evidence in support of many of these therapies is limited. The majority of patients are managed with observation, topical medications, phototherapy, or systemic immunosuppressive therapy. The level of disease activity; depth of involvement; body surface area involved; and the presence of functional impairment, extracutaneous manifestations, or cosmetic disfigurement determine the most appropriate approach to treatment (algorithm 1).

• #2 Morphea – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/diagnosis-treatment/drc-20375290

  Morphea usually lasts several years and then goes away without treatment. It may leave scars or areas of darkened or discolored skin. Until your condition clears up, you may want to pursue treatment that helps control your signs and symptoms. […] Treatment options vary depending on the extent of your condition and how it’s affecting your life. They include: […] Your doctor may prescribe a vitamin D cream, such as calcipotriene, to help soften the skin patches. Skin generally begins to improve during the first months of treatment. Possible side effects include burning, stinging and a rash. […] Or your doctor may prescribe a corticosteroid cream to reduce inflammation. When used for a long time, these creams may thin the skin. […] For severe or widespread morphea, treatment may include the use of ultraviolet light (phototherapy).

• #3 Morphea (localized scleroderma) in adults: Management – UpToDate

  https://www.uptodate.com/contents/morphea-localized-scleroderma-in-adults-management

  Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Affected patients present with single or multiple inflammatory and sclerotic plaques, findings considered manifestations of active disease. These plaques eventually become inactive, leaving permanent dermal or soft tissue atrophy and pigmentary changes (skin damage). […] There are multiple treatment options for active morphea; however, evidence in support of many of these therapies is limited. The majority of patients are managed with observation, topical medications, phototherapy, or systemic immunosuppressive therapy. The level of disease activity; depth of involvement; body surface area involved; and the presence of functional impairment, extracutaneous manifestations, or cosmetic disfigurement determine the most appropriate approach to treatment (algorithm 1).

• #4 Morphea (localized scleroderma) in adults: Management – UpToDate

  https://www.uptodate.com/contents/treatment-of-morphea-localized-scleroderma-in-adults

  Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Affected patients present with single or multiple inflammatory and sclerotic plaques, findings considered manifestations of active disease. […] There are multiple treatment options for active morphea; however, evidence in support of many of these therapies is limited. The majority of patients are managed with observation, topical medications, phototherapy, or systemic immunosuppressive therapy. […] Systemic therapy is often warranted for patients with extensive active morphea that is rapidly progressing, causing significant cosmetic disfigurement, or is accompanied by extracutaneous manifestations. […] The management of morphea in adults will be reviewed here.

• #5 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression. […] Topical therapy may help limited and superficial forms of morphoea and can reduce the itch. […] Phototherapy can soften morphoea and has anti-inflammatory effects. […] Systemic treatment is intended to prevent progression and switch off the active disease process. […] Specific treatment decisions in morphoea are guided by the subtype of morphoea and its severity. […] Physiotherapy to improve joint mobility should be undertaken with caution in active morphoea, as the resultant trauma may potentially act as an ongoing disease trigger. […] In some cases, surgery may be of benefit, such as autologous fat transfer, to improve atrophy. […] Disease progression and treatment response can be monitored using photographs, the localized scleroderma cutaneous assessment tool(LoSCAT) and other highly specialised tests, such as infrared thermography. […] Extended courses of systemic treatments of 4 to 5 years or more may be required to minimise the risk of relapse.

• #6 Delays in Diagnosis, Variations in Treatment for Morphea – MPR

  https://www.empr.com/home/news/drug-news/delays-in-diagnosis-variations-in-treatment-for-morphea/

  Patients with localized scleroderma (morphea) often experience delay in diagnosis and variability in treatment that is based more on the specialty of the provider than disease characteristics, according to a study published in the November issue of the Journal of the American Academy of Dermatology. […] The researchers found that 63 percent of patients received a diagnosis more than six months after morphea onset. […] Topical corticosteroids were the most commonly prescribed therapy (63 percent). […] Dermatologists mainly prescribed topical treatments or phototherapy, even for those with linear and generalized morphea, while rheumatologists mainly prescribed systemic immunosuppressives and physical therapy. […] In summary, we identified several factors that impact the care of patients with morphea, the authors write. These include delay in diagnosis and treatment, large variation in evaluation and therapy based on the specialty of the provider, and widespread use of evaluations and therapy with little evidence for their efficacy.

• #7 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Morphea, also called localized scleroderma, is a rare inflammatory skin condition that can also affect the subcutaneous tissues. […] This activity reviews the evaluation and treatment of morphea and highlights the role of the interprofessional team in caring for patients with this condition. […] Summarize the treatment of morphea. […] Clinicians should review the subtype of morphea, the depth of involvement, and disease activity, which play a significant role in treatment decisions. Early diagnosis and treatment are necessary to minimize damage such as cosmetic sequelae and joint contractures or limb deformities in patients with severe forms of morphea. […] In patients with superficial circumscribed lesions, topical treatments are appropriate and offer an excellent therapeutic response. Topical corticosteroids are first-line treatment for superficial morphea, and the usual treatment is for 3-4 weeks. Topical tacrolimus 0.1% is an alternate choice for superficial circumscribed morphea.

• #8 Morphea – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/diagnosis-treatment/drc-20375290

  Morphea usually lasts several years and then goes away without treatment. It may leave scars or areas of darkened or discolored skin. Until your condition clears up, you may want to pursue treatment that helps control your signs and symptoms. […] Treatment options vary depending on the extent of your condition and how it’s affecting your life. They include: […] Your doctor may prescribe a vitamin D cream, such as calcipotriene, to help soften the skin patches. Skin generally begins to improve during the first months of treatment. Possible side effects include burning, stinging and a rash. […] Or your doctor may prescribe a corticosteroid cream to reduce inflammation. When used for a long time, these creams may thin the skin. […] For severe or widespread morphea, treatment may include the use of ultraviolet light (phototherapy).

• #9 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Although several regimens have shown benefit in case series, few controlled trials have been performed and data suggest wide variation in the approach to treatment. […] In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions. […] Limited disease can often be managed with topical therapy or lesion-limited phototherapy. […] Treatment of active lesions with superpotent topical or intralesional corticosteroids may help reduce inflammation and prevent progression, although there is a lack of data supporting their efficacy. […] A typical regimen includes alternating every other day a super-potent topical steroid with a steroid-sparing agent, such as tacrolimus or calcipotriene. […] Tacrolimus 0.1% ointment applied twice daily was shown to be effective as monotherapy for limited plaque morphea compared with placebo in a 12-week, small, double-blind, placebo-controlled study.

• #10 Morphea Medication: Corticosteroids, Vitamin D analogs, Antirheumatic agents, Immunosuppressant agents

  https://emedicine.medscape.com/article/1065782-medication

  The mechanism of action of tacrolimus is not known. It reduces itching and inflammation by suppressing the release of cytokines from T cells. […] Mycophenolate inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses denovo purine synthesis by lymphocytes, thereby inhibiting their proliferation. It inhibits antibody production. […] Two formulations are available and are not interchangeable. […] Calcipotriene is a synthetic vitamin D-3 analog that can lead to softening of morphea lesions. […] Treatment of pediatric localized scleroderma with methotrexate. […] Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea. […] Treatment of Generalized Deep Morphea With Everolimus. […] A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma.

• #11 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. […] The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective. […] Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series. […] Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy. […] Successful treatment of severe and/or rapidly progressive morphea with systemic corticosteroids (eg, high-dose intravenous methylprednisolone in monthly pulses or oral prednisone at various intervals) in combination with weekly low-dose methotrexate (MTX) has been reported in several case series.

• #12 Morphea Medication: Corticosteroids, Vitamin D analogs, Antirheumatic agents, Immunosuppressant agents

  https://emedicine.medscape.com/article/1065782-medication

  The mechanism of action of tacrolimus is not known. It reduces itching and inflammation by suppressing the release of cytokines from T cells. […] Mycophenolate inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses denovo purine synthesis by lymphocytes, thereby inhibiting their proliferation. It inhibits antibody production. […] Two formulations are available and are not interchangeable. […] Calcipotriene is a synthetic vitamin D-3 analog that can lead to softening of morphea lesions. […] Treatment of pediatric localized scleroderma with methotrexate. […] Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea. […] Treatment of Generalized Deep Morphea With Everolimus. […] A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma.

• #13 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. […] The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective. […] Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series. […] Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy. […] Successful treatment of severe and/or rapidly progressive morphea with systemic corticosteroids (eg, high-dose intravenous methylprednisolone in monthly pulses or oral prednisone at various intervals) in combination with weekly low-dose methotrexate (MTX) has been reported in several case series.

• #14 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. […] The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective. […] Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series. […] Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy. […] Successful treatment of severe and/or rapidly progressive morphea with systemic corticosteroids (eg, high-dose intravenous methylprednisolone in monthly pulses or oral prednisone at various intervals) in combination with weekly low-dose methotrexate (MTX) has been reported in several case series.

• #15 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression. […] Topical therapy may help limited and superficial forms of morphoea and can reduce the itch. […] Phototherapy can soften morphoea and has anti-inflammatory effects. […] Systemic treatment is intended to prevent progression and switch off the active disease process. […] Specific treatment decisions in morphoea are guided by the subtype of morphoea and its severity. […] Physiotherapy to improve joint mobility should be undertaken with caution in active morphoea, as the resultant trauma may potentially act as an ongoing disease trigger. […] In some cases, surgery may be of benefit, such as autologous fat transfer, to improve atrophy. […] Disease progression and treatment response can be monitored using photographs, the localized scleroderma cutaneous assessment tool(LoSCAT) and other highly specialised tests, such as infrared thermography. […] Extended courses of systemic treatments of 4 to 5 years or more may be required to minimise the risk of relapse.

• #16 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  In one case report, treatment of generalized morphea with concomitant mycophenolate and intravenous immunoglobulin (IVIG) resulted in significant improvement. […] A few reports describe responses of severe morphea to cyclosporine and everolimus. […] The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature. […] However, a retrospective study of 84 adults with morphea treated with at least 6 months of hydroxychloroquine showed 43% of patients had a complete response, with only 7.1% having no response. […] Despite promising results in case series involving both adults and children, oral calcitriol did not lead to significant improvement in a double-blinded placebo-controlled trial. […] Phototherapy can also be considered a first-line therapy for patients with generalized, superficial morphea given its low adverse effect profile compared with immunosuppressive agents.

• #17 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  There are many types of phototherapy, including narrowband UVB (310-311 nm), broadband UVB, broadband UVA (320-400 nm), long-wavelength UVA (UVA1; 340-400 nm, low- or medium-dose), and psoralen plus UVA chemotherapy (PUVA). […] All modalities have been reported to be beneficial, with UVA being the most studied to date. […] UVA-based phototherapy modalities (broadband UVA, UVA1, PUVAboth oral and bath) have all been shown to improve morphea lesions in multiple case series and a randomized controlled trial. […] PUVA (both bath and systemic) was found to be effective in a small uncontrolled study and case series. […] A combination of acitretin and PUVA has also shown efficacy. […] In a randomized trial comparing the efficacy of different forms of phototherapy for morphea, medium dose UVA1 (50 J/cm2) was found to be significantly more effective than narrowband UVB.

• #18 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  There are many types of phototherapy, including narrowband UVB (310-311 nm), broadband UVB, broadband UVA (320-400 nm), long-wavelength UVA (UVA1; 340-400 nm, low- or medium-dose), and psoralen plus UVA chemotherapy (PUVA). […] All modalities have been reported to be beneficial, with UVA being the most studied to date. […] UVA-based phototherapy modalities (broadband UVA, UVA1, PUVAboth oral and bath) have all been shown to improve morphea lesions in multiple case series and a randomized controlled trial. […] PUVA (both bath and systemic) was found to be effective in a small uncontrolled study and case series. […] A combination of acitretin and PUVA has also shown efficacy. […] In a randomized trial comparing the efficacy of different forms of phototherapy for morphea, medium dose UVA1 (50 J/cm2) was found to be significantly more effective than narrowband UVB.

• #19 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  There are many types of phototherapy, including narrowband UVB (310-311 nm), broadband UVB, broadband UVA (320-400 nm), long-wavelength UVA (UVA1; 340-400 nm, low- or medium-dose), and psoralen plus UVA chemotherapy (PUVA). […] All modalities have been reported to be beneficial, with UVA being the most studied to date. […] UVA-based phototherapy modalities (broadband UVA, UVA1, PUVAboth oral and bath) have all been shown to improve morphea lesions in multiple case series and a randomized controlled trial. […] PUVA (both bath and systemic) was found to be effective in a small uncontrolled study and case series. […] A combination of acitretin and PUVA has also shown efficacy. […] In a randomized trial comparing the efficacy of different forms of phototherapy for morphea, medium dose UVA1 (50 J/cm2) was found to be significantly more effective than narrowband UVB.

• #20 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  However, there was no difference in clinical efficacy between narrowband UVB and low-dose UVA1 (20 J/cm2) or between medium- and low-dose UVA1. […] Despite these findings, the use of UVA1 is limited as only some academic centers have UVA1 units. […] Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial and is the most commonly used phototherapy modality for patients. […] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. […] Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. […] In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. […] Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea.

• #21 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  However, there was no difference in clinical efficacy between narrowband UVB and low-dose UVA1 (20 J/cm2) or between medium- and low-dose UVA1. […] Despite these findings, the use of UVA1 is limited as only some academic centers have UVA1 units. […] Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial and is the most commonly used phototherapy modality for patients. […] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. […] Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. […] In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. […] Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea.

• #22 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  However, there was no difference in clinical efficacy between narrowband UVB and low-dose UVA1 (20 J/cm2) or between medium- and low-dose UVA1. […] Despite these findings, the use of UVA1 is limited as only some academic centers have UVA1 units. […] Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial and is the most commonly used phototherapy modality for patients. […] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. […] Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. […] In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. […] Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea.

• #23 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  However, there was no difference in clinical efficacy between narrowband UVB and low-dose UVA1 (20 J/cm2) or between medium- and low-dose UVA1. […] Despite these findings, the use of UVA1 is limited as only some academic centers have UVA1 units. […] Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial and is the most commonly used phototherapy modality for patients. […] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. […] Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. […] In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. […] Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea.

• #24 Morphea (localized scleroderma) in adults: Management – UpToDate

  https://www.uptodate.com/contents/morphea-localized-scleroderma-in-adults-management

  Systemic therapy is often warranted for patients with extensive active morphea that is rapidly progressing, causing significant cosmetic disfigurement, or is accompanied by extracutaneous manifestations. Occupational and physical therapy are important interventions for patients who have or are at risk for disability.

• #25 Morphea (localized scleroderma) in adults: Management – UpToDate

  https://www.uptodate.com/contents/treatment-of-morphea-localized-scleroderma-in-adults

  Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Affected patients present with single or multiple inflammatory and sclerotic plaques, findings considered manifestations of active disease. […] There are multiple treatment options for active morphea; however, evidence in support of many of these therapies is limited. The majority of patients are managed with observation, topical medications, phototherapy, or systemic immunosuppressive therapy. […] Systemic therapy is often warranted for patients with extensive active morphea that is rapidly progressing, causing significant cosmetic disfigurement, or is accompanied by extracutaneous manifestations. […] The management of morphea in adults will be reviewed here.

• #26 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. […] The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective. […] Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series. […] Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy. […] Successful treatment of severe and/or rapidly progressive morphea with systemic corticosteroids (eg, high-dose intravenous methylprednisolone in monthly pulses or oral prednisone at various intervals) in combination with weekly low-dose methotrexate (MTX) has been reported in several case series.

• #27 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #28 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #29 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #30 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #31 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #32 Update on Management of Morphea (Localized Scleroderma) in Children

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7247622/

  Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. The assessment of activity and damage can be done with a validated instrument like LoSCAT. Disease classified as low severity which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. Methotrexate is recommended for linear, deep and generalized morphea. Steroids are effective in the early inflammatory stage and used in combination with methotrexate. Methotrexate is continued for at least 12 months after adequate response is achieved. Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate. There are also reports of improvement of disease with ciclosporine and hydroxychloroquine. In severe cases, recalcitrant to standard therapy there may be a role for biologics, JAK inhibitors, and IVIG. Supportive measures like physiotherapy and psychiatric counseling are also important in the management of morphea. Orthopedic surgery and other measures like autologous fat transfer may be advocated once the disease is inactive.

• #33 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. […] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. […] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. […] Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation. […] Mycophenolate mofetil has been shown to be an alternative agent to methotrexate. […] It is believed to function through antifibrotic mechanisms.

• #34 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  In one case report, treatment of generalized morphea with concomitant mycophenolate and intravenous immunoglobulin (IVIG) resulted in significant improvement. […] A few reports describe responses of severe morphea to cyclosporine and everolimus. […] The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature. […] However, a retrospective study of 84 adults with morphea treated with at least 6 months of hydroxychloroquine showed 43% of patients had a complete response, with only 7.1% having no response. […] Despite promising results in case series involving both adults and children, oral calcitriol did not lead to significant improvement in a double-blinded placebo-controlled trial. […] Phototherapy can also be considered a first-line therapy for patients with generalized, superficial morphea given its low adverse effect profile compared with immunosuppressive agents.

• #35 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  In one case report, treatment of generalized morphea with concomitant mycophenolate and intravenous immunoglobulin (IVIG) resulted in significant improvement. […] A few reports describe responses of severe morphea to cyclosporine and everolimus. […] The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature. […] However, a retrospective study of 84 adults with morphea treated with at least 6 months of hydroxychloroquine showed 43% of patients had a complete response, with only 7.1% having no response. […] Despite promising results in case series involving both adults and children, oral calcitriol did not lead to significant improvement in a double-blinded placebo-controlled trial. […] Phototherapy can also be considered a first-line therapy for patients with generalized, superficial morphea given its low adverse effect profile compared with immunosuppressive agents.

• #36 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  In one case report, treatment of generalized morphea with concomitant mycophenolate and intravenous immunoglobulin (IVIG) resulted in significant improvement. […] A few reports describe responses of severe morphea to cyclosporine and everolimus. […] The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature. […] However, a retrospective study of 84 adults with morphea treated with at least 6 months of hydroxychloroquine showed 43% of patients had a complete response, with only 7.1% having no response. […] Despite promising results in case series involving both adults and children, oral calcitriol did not lead to significant improvement in a double-blinded placebo-controlled trial. […] Phototherapy can also be considered a first-line therapy for patients with generalized, superficial morphea given its low adverse effect profile compared with immunosuppressive agents.

• #37 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  However, there was no difference in clinical efficacy between narrowband UVB and low-dose UVA1 (20 J/cm2) or between medium- and low-dose UVA1. […] Despite these findings, the use of UVA1 is limited as only some academic centers have UVA1 units. […] Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial and is the most commonly used phototherapy modality for patients. […] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. […] Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. […] In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. […] Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea.

• #38 Scientists Identify Treatment Option for Rare Pansclerotic Morphea

  https://www.dermatologytimes.com/view/scientists-identify-treatment-option-for-rare-pansclerotic-morphea

  Scientists have found a new therapy for the severe inflammatory skin disorder. […] Scientists affiliated with the National Institutes of Health (NIH) and their colleagues have identified a potential treatment option for disabling pansclerotic morphea (DPM). […] Inhibition of Janus kinase (JAK)STAT signaling with ruxolitinib led to improvement in the hyperinflammatory fibroblast phenotype in vitro and resolution of inflammatory markers and clinical symptoms in treated patients, without adverse effects. […] Researchers said they hope to continue studying molecules within this pathway in order to make further advancements for patients with DPM and related conditions. […] Kastner said these results play a role in the future of treating patients with wound healing and scarring conditions. […] The findings of this study open doors for JAK inhibitors to be a potential treatment for other inflammatory skin disorders or disorders related to tissue scarring, whether it is scarring of the lungs, liver or bone marrow.

• #39 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Other approaches aim to alter the inflammatory milieu. […] These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, biologic drugs, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). […] Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. […] Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. […] Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. […] Such surgical interventions include release of joint contractures and limb-lengthening procedures.

• #40 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Other approaches aim to alter the inflammatory milieu. […] These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, biologic drugs, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). […] Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. […] Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. […] Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. […] Such surgical interventions include release of joint contractures and limb-lengthening procedures.

• #41 AAD Reading Room | Can Tocilizumab Deep Six Recalcitrant Morphea? | MedPage Today

  https://www.medpagetoday.com/reading-room/aad/general-dermatology/107443

  Warren R. Heymann, MD, FAAD […] Tocilizumab has recently been reported to be beneficial in patients with the following morphea variants that were refractory to standard therapies (all are case reports or small case series): juvenile localized scleroderma, pansclerotic morphea, ECDS and Parry Romberg, and checkpoint inhibitor-induced generalized morphea. Tocilizumab may be effective in adults with recalcitrant morphea as well as children. […] Tocilizumab is generally well-tolerated. Common adverse effects include infections (cellulitis, urinary tract, and respiratory tract), gastrointestinal (nausea, vomiting, diarrhea, elevated transaminases, and rarely lower intestinal bowel perforation), and hyperlipidemia (cholesterol and triglycerides). […] In conclusion, tocilizumab may be a valuable therapeutic option for patients with morphea recalcitrant to standard treatments.

• #42 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Other approaches aim to alter the inflammatory milieu. […] These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, biologic drugs, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). […] Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. […] Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. […] Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. […] Such surgical interventions include release of joint contractures and limb-lengthening procedures.

• #43 Morphea (Scleroderma): Treatment, Causes, and More

  https://www.verywellhealth.com/morphea-5179411

  Phototherapy is also an option for these people. UVA or narrowband UVB can be used. This may include using a medication called psoralen (either taken orally or applied to the skin through a bath). […] Oral medications that affect the immune system might be needed as well, particularly if phototherapy isnt an option. […] Other potential treatments for morphea are currently under investigation, but we dont know as much about their use. […] Physical therapy and occupational therapy can be key for people who have joint contractures or deeper muscle and joint involvement. […] Typically, surgery isnt recommended in the initial inflammatory part of the disease but only after the condition is medically under control.

• #44 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Dermatologic and plastic surgeons can help correct deformities due to atrophy of subcutaneous tissues. […] Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials. […] Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea, as there are demonstrated gaps in care with a wide range of approaches. […] Consultation with a physical and occupational therapist and a program of physical therapy are of utmost importance in maintaining range of motion and function of the extremities in patients with linear or deep morphea that crosses joint lines. […] Programs typically include passive stretching, muscle strengthening, and resting splints.

• #45 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Dermatologic and plastic surgeons can help correct deformities due to atrophy of subcutaneous tissues. […] Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials. […] Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea, as there are demonstrated gaps in care with a wide range of approaches. […] Consultation with a physical and occupational therapist and a program of physical therapy are of utmost importance in maintaining range of motion and function of the extremities in patients with linear or deep morphea that crosses joint lines. […] Programs typically include passive stretching, muscle strengthening, and resting splints.

• #46 Morphea – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/diagnosis-treatment/drc-20375290

  For severe or widespread morphea, your doctor may prescribe an immunosuppressive medication, such as oral methotrexate (Trexall), corticosteroid pills or both. Or your doctor may suggest hydroxychloroquine (Plaquenil) or mycophenolate mofetil. Each of these drugs has potential side effects. Talk with your doctor about a drug’s risks and benefits before using it. […] If the condition affects your joints, physical therapy might preserve your range of motion.

• #47 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Other approaches aim to alter the inflammatory milieu. […] These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, biologic drugs, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). […] Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. […] Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. […] Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. […] Such surgical interventions include release of joint contractures and limb-lengthening procedures.

• #48 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Other approaches aim to alter the inflammatory milieu. […] These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, biologic drugs, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). […] Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. […] Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. […] Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. […] Such surgical interventions include release of joint contractures and limb-lengthening procedures.

• #49 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Dermatologic and plastic surgeons can help correct deformities due to atrophy of subcutaneous tissues. […] Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials. […] Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea, as there are demonstrated gaps in care with a wide range of approaches. […] Consultation with a physical and occupational therapist and a program of physical therapy are of utmost importance in maintaining range of motion and function of the extremities in patients with linear or deep morphea that crosses joint lines. […] Programs typically include passive stretching, muscle strengthening, and resting splints.

• #50 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Dermatologic and plastic surgeons can help correct deformities due to atrophy of subcutaneous tissues. […] Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials. […] Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea, as there are demonstrated gaps in care with a wide range of approaches. […] Consultation with a physical and occupational therapist and a program of physical therapy are of utmost importance in maintaining range of motion and function of the extremities in patients with linear or deep morphea that crosses joint lines. […] Programs typically include passive stretching, muscle strengthening, and resting splints.

• #51 Morphea – Treatments | SRUK

  https://www.sruk.co.uk/scleroderma/scleroderma-treatments/morphea-treatments/

  Physical therapy. This type of treatment uses exercise to prevent joint deformity and maintain movement. […] If any contractures of joints occur, surgery and long-term physiotherapy can be of use. Plastic surgery may be a possible form of treatment for deeper forms of morphoea, in particular en coup de sabre. At all times, the side effects of treatment versus the condition itself must be weighed up by the patient and his or her doctor. If the disease is mild or not progressing then it may be appropriate not to apply or take specific therapy. In extensive morphoea or when there is interference with growth, as can occur in childhood, then treatment should be much more intensive.

• #52 Update on Management of Morphea (Localized Scleroderma) in Children

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7247622/

  Mycophenolate mofetil has been found to be effective in the treatment resistant JLS. […] Hydroxychloroquine (HCQ) has been shown to be effective in the treatment of morphea in a retrospective study of 84 patients between the ages of 477 years. […] Ciclosporine has shown the drug to be effective. […] The evidence for the use of biologics and small molecules is based on case reports. […] Phototherapy/Photochemotherapy has been used in the treatment of JLS. […] The current recommendation by experts is to use phototherapy, UVA1 or narrow band UVB, preferably for children 12 years with superficial, non-progressive morphea that does not cross joints or occurring in non-cosmetically sensitive areas. […] Reconstructive surgery including autologous fat transfers, and injection of fillers for correction of cosmetic problems in en coup de sabre form of localized scleroderma can be done once the disease is inactive.

• #53

  https://scholars.duke.edu/publication/1351741

  Use of oral calcipotriol, D-penicillamine, interferon gamma, and antimalarials is not supported. […] Phototherapy, methotrexate/systemic corticosteroids, calcipotriene, and topical tacrolimus have the most evidence for efficacy in morphea. […] Treatment works best in inflammatory disease. […] Disease activity, severity, progression, and depth should play a role in therapeutic decision making.

• #54 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Type of treatment is based on the type of localised scleroderma and its extent – for example: […] Limited superficial plaque morphoea may respond to topical treatment or local phototherapy. […] Deep plaque morphoea may require phototherapy or systemic immunosuppression with methotrexate and steroids. […] Linear morphoea is usually aggressively treated with immunosuppression. […] Generalised morphoea is usually not suitable for topical therapy, due to the large surface area involved, so phototherapy or immunosuppression is usually required.

• #55 Morphea: Evidence-based recommendations for treatment – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/morphea-evidence-based-recommendations-for-treatment/

  This suggests that twice-daily occluded calcipotriene is an effective therapy for active plaque or linear morphea. […] This suggests that interferon gamma is not an effective therapy for morphea. […] This suggests that oral calcitriol is not an effective treatment for morphea. […] This study suggests that low-dose UVA1, medium-dose UVA1 and narrow-band UVB may all be effective therapies for morphea. […] The combination of oral prednisone and methotrexate is an effective therapy for active morphea. […] These three prospective studies suggest that treatment of morphea with systemic methotrexate and steroids is effective. […] These studies suggest that broadband UVA therapy and PUVA may be effective treatments for morphea. […] Patients with limited plaque morphea should be primarily treated with topical tacrolimus twice daily.

• #56 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Localised scleroderma treatment and management […] Unfortunately there is no available, effective treatment for most cases of localised scleroderma. As no truly effective treatment yet exists for localised scleroderma, management must be based on the extent and the severity of the disease, and focus primarily on the risk of deformities and movement limitation. Physiotherapy may be helpful to prevent joint contractures in severe disease. There is evidence for efficacy of the following treatment options: […] Ultraviolet B (UVB) phototherapy for superficial skin involvement. […] UVA phototherapy for deeper skin involvement. Efficacy has been proven, although there is no certainty yet about the long-term safety and the risk of skin cancer. […] Immunosuppression. The best evidence is for methotrexate +/- systemic corticosteroids for involvement of underlying tissues and for rapidly progressive or severe disease.

• #57 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Type of treatment is based on the type of localised scleroderma and its extent – for example: […] Limited superficial plaque morphoea may respond to topical treatment or local phototherapy. […] Deep plaque morphoea may require phototherapy or systemic immunosuppression with methotrexate and steroids. […] Linear morphoea is usually aggressively treated with immunosuppression. […] Generalised morphoea is usually not suitable for topical therapy, due to the large surface area involved, so phototherapy or immunosuppression is usually required.

• #58 Morphea: Evidence-based recommendations for treatment – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/morphea-evidence-based-recommendations-for-treatment/

  Based on the available evidence, methotrexate in combination with a short course of systemic steroids is first-line therapy. […] If the patient has not improved after 2-3 months, then switching therapy to methotrexate in combination with systemic steroids is a next step. […] In conclusion, additional trials of therapeutic options for patients with morphea are needed.

• #59 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Localised scleroderma treatment and management […] Unfortunately there is no available, effective treatment for most cases of localised scleroderma. As no truly effective treatment yet exists for localised scleroderma, management must be based on the extent and the severity of the disease, and focus primarily on the risk of deformities and movement limitation. Physiotherapy may be helpful to prevent joint contractures in severe disease. There is evidence for efficacy of the following treatment options: […] Ultraviolet B (UVB) phototherapy for superficial skin involvement. […] UVA phototherapy for deeper skin involvement. Efficacy has been proven, although there is no certainty yet about the long-term safety and the risk of skin cancer. […] Immunosuppression. The best evidence is for methotrexate +/- systemic corticosteroids for involvement of underlying tissues and for rapidly progressive or severe disease.

• #60 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Type of treatment is based on the type of localised scleroderma and its extent – for example: […] Limited superficial plaque morphoea may respond to topical treatment or local phototherapy. […] Deep plaque morphoea may require phototherapy or systemic immunosuppression with methotrexate and steroids. […] Linear morphoea is usually aggressively treated with immunosuppression. […] Generalised morphoea is usually not suitable for topical therapy, due to the large surface area involved, so phototherapy or immunosuppression is usually required.

• #61 Morphoea (Localised Scleroderma) | Doctor

  https://patient.info/doctor/localised-scleroderma-morphoea-pro

  Type of treatment is based on the type of localised scleroderma and its extent – for example: […] Limited superficial plaque morphoea may respond to topical treatment or local phototherapy. […] Deep plaque morphoea may require phototherapy or systemic immunosuppression with methotrexate and steroids. […] Linear morphoea is usually aggressively treated with immunosuppression. […] Generalised morphoea is usually not suitable for topical therapy, due to the large surface area involved, so phototherapy or immunosuppression is usually required.

• #62 Update on Management of Morphea (Localized Scleroderma) in Children

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7247622/

  Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. The assessment of activity and damage can be done with a validated instrument like LoSCAT. Disease classified as low severity which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. Methotrexate is recommended for linear, deep and generalized morphea. Steroids are effective in the early inflammatory stage and used in combination with methotrexate. Methotrexate is continued for at least 12 months after adequate response is achieved. Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate. There are also reports of improvement of disease with ciclosporine and hydroxychloroquine. In severe cases, recalcitrant to standard therapy there may be a role for biologics, JAK inhibitors, and IVIG. Supportive measures like physiotherapy and psychiatric counseling are also important in the management of morphea. Orthopedic surgery and other measures like autologous fat transfer may be advocated once the disease is inactive.

• #63 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression. […] Topical therapy may help limited and superficial forms of morphoea and can reduce the itch. […] Phototherapy can soften morphoea and has anti-inflammatory effects. […] Systemic treatment is intended to prevent progression and switch off the active disease process. […] Specific treatment decisions in morphoea are guided by the subtype of morphoea and its severity. […] Physiotherapy to improve joint mobility should be undertaken with caution in active morphoea, as the resultant trauma may potentially act as an ongoing disease trigger. […] In some cases, surgery may be of benefit, such as autologous fat transfer, to improve atrophy. […] Disease progression and treatment response can be monitored using photographs, the localized scleroderma cutaneous assessment tool(LoSCAT) and other highly specialised tests, such as infrared thermography. […] Extended courses of systemic treatments of 4 to 5 years or more may be required to minimise the risk of relapse.

• #64 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression. […] Topical therapy may help limited and superficial forms of morphoea and can reduce the itch. […] Phototherapy can soften morphoea and has anti-inflammatory effects. […] Systemic treatment is intended to prevent progression and switch off the active disease process. […] Specific treatment decisions in morphoea are guided by the subtype of morphoea and its severity. […] Physiotherapy to improve joint mobility should be undertaken with caution in active morphoea, as the resultant trauma may potentially act as an ongoing disease trigger. […] In some cases, surgery may be of benefit, such as autologous fat transfer, to improve atrophy. […] Disease progression and treatment response can be monitored using photographs, the localized scleroderma cutaneous assessment tool(LoSCAT) and other highly specialised tests, such as infrared thermography. […] Extended courses of systemic treatments of 4 to 5 years or more may be required to minimise the risk of relapse.

• #65 Morphea: Effective Treatment with Homeopathy –

  https://ndnr.com/cardiopulmonary-medicine/morphea-effective-treatment-with-homeopathy/

  Morphea, also known as localized scleroderma, is a rare disease with an incidence rate of 3 in 100 000 and a prevalence rate of 50 per 100 000. […] Current first-line treatments with the most evidence for efficacy in morphea include phototherapy, methotrexate, systemic corticosteroids, calcipotriene, and topical tacrolimus. […] However, there is no consensus on the treatment of morphea, and most of the studies are case series, with very few comparative or placebo-controlled clinical trials. […] Management of morphea remains unsatisfactory or inconsistent, as some patients receive systemic immunosuppressives, while others receive little to no therapy. […] Methotrexate, either alone or with corticosteroids, has the greatest level of evidence. […] However, recurrence rates following discontinuation of treatment are high, ranging from 30-40%.

• #66

  https://scholars.duke.edu/publication/1351741

  Use of oral calcipotriol, D-penicillamine, interferon gamma, and antimalarials is not supported. […] Phototherapy, methotrexate/systemic corticosteroids, calcipotriene, and topical tacrolimus have the most evidence for efficacy in morphea. […] Treatment works best in inflammatory disease. […] Disease activity, severity, progression, and depth should play a role in therapeutic decision making.

• #67 Morphea Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1065782-treatment

  Although several regimens have shown benefit in case series, few controlled trials have been performed and data suggest wide variation in the approach to treatment. […] In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions. […] Limited disease can often be managed with topical therapy or lesion-limited phototherapy. […] Treatment of active lesions with superpotent topical or intralesional corticosteroids may help reduce inflammation and prevent progression, although there is a lack of data supporting their efficacy. […] A typical regimen includes alternating every other day a super-potent topical steroid with a steroid-sparing agent, such as tacrolimus or calcipotriene. […] Tacrolimus 0.1% ointment applied twice daily was shown to be effective as monotherapy for limited plaque morphea compared with placebo in a 12-week, small, double-blind, placebo-controlled study.

• #68 Morphea Medication: Corticosteroids, Vitamin D analogs, Antirheumatic agents, Immunosuppressant agents

  https://emedicine.medscape.com/article/1065782-medication

  In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions. […] The approach to treatment of the various subtypes of morphea is described in Medical Care. Note that phototherapy represents another important modality. […] Prednisone reduces inflammation and prevents the progression of sclerosis. Systemic corticosteroid therapy (often used in combination with MTX; see below) is appropriate for patients with active inflammatory disease that is widespread, severe, and/or potentially disfiguring/disabling. […] Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA and RNA synthesis in lymphocytes and other immune cells. This and other mechanisms lead to an anti-inflammatory effect, which is reflected in reduced levels of circulating cytokines such as IL-2, IL-6, and IL-8 (indicators of disease activity) in morphea patients. Response is often delayed until 1-3 months after initiation of therapy.

• #69 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Morphea treatment depends on morphea subtype. […] Most patients with circumscribed morphea will do very well with medicated creams. […] Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation. […] Two main type of phototherapy have been found to be helpful for the itch in systemic sclerosis, narrow band UVB and UVA1. […] Phototherapy generally requires 2 to 3 visits per week over the course of 2 to 3 months. […] Pills that calm the immune system, like methotrexate, mycophenolate mofetil, prednisone, hydroxychloroquine, and other medications, may be used. […] All patients will have less itching and symptoms if the morphea lesions are kept moisturized. […] It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms. […] Sun exposure may be helpful for morphea lesions, but do not over do it. […] Ten to 15 minutes of exposure to midday sunlight two to three times per week may improve the morphea lesions.

• #70 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Morphea treatment depends on morphea subtype. […] Most patients with circumscribed morphea will do very well with medicated creams. […] Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation. […] Two main type of phototherapy have been found to be helpful for the itch in systemic sclerosis, narrow band UVB and UVA1. […] Phototherapy generally requires 2 to 3 visits per week over the course of 2 to 3 months. […] Pills that calm the immune system, like methotrexate, mycophenolate mofetil, prednisone, hydroxychloroquine, and other medications, may be used. […] All patients will have less itching and symptoms if the morphea lesions are kept moisturized. […] It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms. […] Sun exposure may be helpful for morphea lesions, but do not over do it. […] Ten to 15 minutes of exposure to midday sunlight two to three times per week may improve the morphea lesions.

• #71 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Morphea treatment depends on morphea subtype. […] Most patients with circumscribed morphea will do very well with medicated creams. […] Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation. […] Two main type of phototherapy have been found to be helpful for the itch in systemic sclerosis, narrow band UVB and UVA1. […] Phototherapy generally requires 2 to 3 visits per week over the course of 2 to 3 months. […] Pills that calm the immune system, like methotrexate, mycophenolate mofetil, prednisone, hydroxychloroquine, and other medications, may be used. […] All patients will have less itching and symptoms if the morphea lesions are kept moisturized. […] It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms. […] Sun exposure may be helpful for morphea lesions, but do not over do it. […] Ten to 15 minutes of exposure to midday sunlight two to three times per week may improve the morphea lesions.

• #72 Morphea – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/symptoms-causes/syc-20375283

  Morphea usually improves on its own over time, though recurrences are common. In the meantime, medications and therapies are available to help treat the skin discoloration and other effects. […] See your doctor if you notice reddish patches of hardening or thickening skin. Early diagnosis and treatment may help slow the development of new patches and allow your doctor to identify and treat complications before they worsen. […] The condition can affect people at any age. It usually appears between the ages of 2 and 14 or in the mid-40s. […] Morphea can cause a number of complications, including movement problems. Morphea that affects the arms or legs can impair joint mobility. […] Treatment of morphea with hydroxychloroquine: A retrospective review of 84 patients at Mayo Clinic, 1996-2013.

• #73 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-morphea-in-childhood-an-update-articulo-S157821901830088X

  Morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentan, and topical imiquimod 5%. […] There are numerous options for the treatment of morphea, including phototherapy, topical corticosteroids, topical calcipotriol, oral calcitriol, topical tacrolimus, systemic corticosteroids, methotrexate, mycophenolate mofetil, intralesional gamma interferon, ciclosporin A, D-penicillamine, imiquimod, bosentan, infliximab, etanercept, adalimumab, hydroxychloroquine, and photopheresis. […] The treatments for children supported by the highest quality of evidence (based on epidemiological studies) are phototherapy and the PCMT regimen. The form of phototherapy used in most of the studies in the literature was low-dose UV-A1, an option not readily available in Spain. However, 1 study found no differences between low-dose UV-A1 and narrowband UV-B. However, long-term outcomes in patients treated with phototherapy have not been documented. […] In summary, PCMT has been shown to be effective in the control of morphea in the short and medium term. However, since morphea is a chronic disease characterized by intermittent outbreaks and periods of remission, new treatment strategies and long-term follow-up studies are needed.

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