Morphea Treatment & Management

Updated: Jun 17, 2020
  • Author: Victoria P Werth, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Treatment

Medical Care

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. [55] In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions. [56]

Superficial circumscribed morphea

Lesions of superficial circumscribed morphea often undergo gradual spontaneous resolution over a 3- to 5-year period. 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. [57, 58]

Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. [59] The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective. [60]

Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series. [61, 62, 63]

Generalized, linear, or deep morphea

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. [64] A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. [65] MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children. [66, 67, 68, 69, 70] To minimize the risk of relapse, the recommended treatment duration of MTX is at least 2 years. [71]

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. [52, 72, 73] A 2020 multicenter, retrospective cohort study of 77 morphea patients demonstrated 87% of patients had improvement or stable morphea after 9-12 months. [74]

A few reports describe responses of severe morphea to cyclosporine and everolimus. [75, 76, 77]

The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature. However, in 2019, 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. [78]

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. [79, 80]

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. [81] 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, PUVA—both oral and bath) have all been shown to improve morphea lesions in multiple case series and a randomized controlled trial. [82]

PUVA (both bath and systemic) was found effective in a sm-ll uncontrolled study and case series. [83, 84] A combination of acitretin and PUVA has also shown efficacy. [85]

UVA1, unlike broadband UVA, consists only of the deeper penetrating UVA wavelengths. 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. 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. [86]

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. [86] Regimens combining UV therapy with topical corticosteroids or calcipotriene may also be superior to either method alone. [86, 87]

Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea. [88, 89]

In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement. [90]

In one case report, treatment of generalized morphea with concomitant mycophenolate and intravenous immunoglobulin (IVIG) showed significant improvement. [91]

Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea. It is an endothelin receptor antagonist with vasodilatative and antifibrotic properties.

Other approaches aim to alter the inflammatory milieu but await further study. These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, JAK inhibitors, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer). [92, 93, 94]

A randomized, double-blinded, placebo-controlled trial demonstrated no benefit with intralesional interferon gamma. [95]

Abatacept has been reported effective in treating morphea with deep tissue involvement in a small case series. [96]

Tocilizumab has been reported effective in treating juvenile morphea in patients who did not respond to methotrexate/mycophenolate in small case series and reports. [97, 98]

Next:

Surgical Care

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.

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 (eg, polyethylene). [99, 100, 101]

Previous
Next:

Consultations

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. [102]

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.

Ophthalmologic screening is indicated for children with head and neck lesions and/or concomitant central nervous system involvement. [44]

Consultation with a neurologist is helpful for patients with craniofacial morphea who present with neurologic symptoms or have abnormalities detected via MRI of the brain.

Orthopedics and oral and maxillofacial surgery should be consulted as needed for bony and structural abnormalities. [103]

Consultation with a dentist is required when craniofacial morphea leads to altered dentition or malocclusion.

Previous