Morphea Treatment & Management

  • Author: Jennifer V Nguyen, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 29, 2010
 

Medical Care

Although several regimens have shown benefit in case series, few controlled trials have been performed. In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions.

  • Plaque-type morphea often undergoes gradual spontaneous resolution over a 3- to 5-year period. Treatment of active lesions with superpotent topical or intralesional corticosteroids may help reduce inflammation and prevent progression. Therapy with topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication.[27] Other topical agents shown to decrease lesional erythema and induration in small series of morphea patients include tacrolimus 0.1% ointment (under occlusion) and imiquimod 5% cream.[28, 29, 30]
  • Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy.
    • Systemic corticosteroids can be helpful in the inflammatory phases of morphea and for eosinophilic fasciitis, but they have little benefit for established sclerosis.
    • 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. MTX alone can also be effective.[31, 32, 33, 34, 35]
    • 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.[36, 37] Scattered reports have described responses of severe morphea to second-line systemic agents, including cyclosporine, mycophenolate mofetil, and oral retinoids.[38, 39]
    • The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature.[40]
    • Prolonged treatment (eg, >1 y) with penicillamine, a penicillin breakdown product that inhibits the cross-linking of collagen fibers, has been reported as beneficial in small series; however, its use is limited by adverse effects such as renal toxicity.[41]
  • Broadband UVA (320-400 nm, low-dose), long-wavelength UVA (UVA1; 340-400 nm, low- or medium-dose), and psoralen plus UVA (oral or bath) photochemotherapy have produced marked clinical improvement of morphea lesions in multiple case series and a randomized controlled trial. Because UVA1 wavelengths penetrate deeper into the dermis, this modality is particularly effective in the treatment of morphea. Low-, medium-, and high-dose UVA are all effective. Medium-dose UVA1 provides for better long-term results than low-dose UVA1 in morphea as shown by ultrasound assessment.[26] Unfortunately, the availability of UVA1 is currently limited. Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial. Regimens combining UV therapy with topical corticosteroids or calcipotriene may be superior to either method alone.[42, 43]
  • A combination of acitretin and PUVA has also shown efficacy.[44]
  • Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea.[45, 46]
  • In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement.[47]
  • Photodynamic therapy using topical 5-aminolevulinic acid was also effective in a small series.
  • 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 cytokine milieu but await further study. These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), interferon-gamma, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer).[48]
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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.
  • Plastic surgery can help to 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).
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Consultations

  • Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea.
  • Consultation with a physical 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.
  • An evaluation by an ophthalmologist is indicated for craniofacial morphea that involves the periocular area.
  • 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.
  • Consultation with a dentist is required when craniofacial morphea leads to altered dentition or malocclusion.
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Contributor Information and Disclosures
Author

Jennifer V Nguyen, MD  Resident Physician, Department of Dermatology, Hospital of the University of Pennsylvania

Disclosure: Nothing to disclose.

Coauthor(s)

Victoria P Werth, MD  Professor of Dermatology and Medicine, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, Philadelphia Veterans Affairs Medical Center

Victoria P Werth, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American College of Rheumatology, Medical Dermatology Society, Phi Beta Kappa, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Nicole Fett, MD  Clinical Educator, Department of Dermatology, University of Pennsylvania School of Medicine

Nicole Fett, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American Medical Association, Medical Dermatology Society, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Peter Fritsch, MD  Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria

Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Inflammatory plaque-type morphea on the abdomen, characterized by induration, erythema, and a surrounding lilac ring.
A hyperpigmented band of linear morphea involving the right part of the trunk and thigh.
Linear atrophic depression of an en coup de sabre lesion on the right side of the forehead and the frontal part of the scalp.
Morphea profunda involving the left lower extremity, with thickened, taut, bound-down skin.
Histopathology of mature scleroderma; full-thickness sclerosis of the dermis. Photomicrograph courtesy of Dirk Elston, MD.
 
 
 
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