Morphea 

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

Background

Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both. Morphea is classified into plaque, generalized, linear, and deep subtypes according to the clinical presentation and depth of tissue involvement. Unlike systemic sclerosis, morphea lacks features such as sclerodactyly, Raynaud phenomenon, nailfold capillary changes, telangiectasias, or progressive internal organ involvement. Morphea can present with extracutaneous manifestations, including fever, lymphadenopathy, arthralgias, and central nervous system involvement, and laboratory abnormalities, including eosinophilia, polyclonal hypergammaglobulinemia, and positive antinuclear antibodies.[1, 2, 3]

Although rare, epidemiologic studies suggest 0.9-5.7% of patients with morphea progress to systemic scleroderma.[2] The transition may be marked by the development of Raynaud phenomenon and nailfold capillary changes.

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Pathophysiology

Overproduction of collagen by fibroblasts in affected tissues is common to all forms of morphea, although the mechanism by which these fibroblasts are activated is unknown. Proposed factors involved in the pathogenesis of morphea include endothelial cell injury, immunologic (eg, T lymphocyte) and inflammatory activation, and dysregulation of collagen production. An autoimmune component is supported by the frequent presence of autoantibodies in affected individuals, as well as the association of morphea with other autoimmune diseases, including systemic lupus erythematosus, vitiligo, type 1 diabetes, and myasthenia gravis.[1, 3]

Endothelial cell injury is currently thought to be the inciting event in the pathogenesis of morphea. This injury results in increased levels of adhesion molecules (circulating intercellular adhesion molecule-1, vascular cell adhesion molecule 1, and E-selectin) and fibrogenic T-helper 2 cytokines such as interleukin (IL)–4, IL-6, and transforming growth factor-beta (TGF-beta). These cytokines recruit eosinophils, CD4+ T cells, and macrophages, which are present in early morphea lesions and in eosinophilic fasciitis. These cytokines and growth factors also increase fibroblast proliferation and induce synthesis of excess collagen and connective-tissue growth factor. Connective-tissue growth factor is a soluble mediator that enhances and perpetuates the profibrotic effects of TGF-beta. The ultimate result of the endothelial injury and inflammatory cascade is increased collagen and extracellular matrix deposition.[4, 5, 6, 7, 8]

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Epidemiology

Frequency

United States

The incidence of morphea has been estimated as approximately 25 cases per million population per year. The actual incidence is likely higher because many cases may not come to medical attention. Two thirds of adults with morphea present with plaque-type lesions, with generalized, linear, and deep variants each accounting for approximately 10% of cases. Up to half of all cases of morphea occur in pediatric patients. In this group, linear morphea predominates (two thirds of cases), followed by the plaque (25%) and generalized (5%) subtypes. Of note, as many as half the patients with linear morphea have coexistent plaque-type lesions.

Mortality/Morbidity

Morphea typically has a benign, self-limited course. Survival rates for morphea patients are no different from those of the general population. However, linear and deep morphea subtypes can cause considerable morbidity, especially in children when they interfere with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy result in substantial disability and deformity in a quarter to half of all patients with linear or deep morphea. Neurologic and ophthalmologic manifestations can also occur in those with craniofacial lesions (eg, en coup de sabre, Parry-Romberg syndrome). Such complications are more common in pediatric cases. Depression and anxiety are prevalent in patients with morphea and correlate with the amount of skin involvement.[9]

Race

Although morphea occurs in persons of all races, it appears to be more common in whites than in African Americans.

Sex

Women are affected approximately 3 times as often as men for all forms of morphea except the linear subtype, which only has a slight female predominance.

Age

Linear morphea commonly manifests in children and adolescents, with two thirds of cases occurring before age 18 years. Other morphea subtypes have a peak incidence in the third and fourth decades of life.

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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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