eMedicine Specialties > Dermatology > Connective Tissue Diseases

Morphea

Author: Kendra G Bergstrom, MD, Staff Physician, Ronald O Perelman Department of Dermatology, New York University School of Medicine
Coauthor(s): Julie V Schaffer, MD, Assistant Professor, Departments of Dermatology and Pediatrics, New York University School of Medicine
Contributor Information and Disclosures

Updated: Aug 3, 2006

Introduction

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, and internal organ involvement.

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 etiology is supported by the frequent presence of autoantibodies in affected individuals.

Studies have shown increased levels of circulating intercellular adhesion molecule-1 and fibrogenic T-helper 2 cytokines such as interleukin (IL)–4 and transforming growth factor-beta (TGF-beta) in patients with morphea. These cytokines recruit eosinophils and other inflammatory cells (which are present in early morphea lesions and in eosinophilic fasciitis) and induce fibroblasts to synthesize excessive collagen and connective-tissue growth factor. The latter is a soluble mediator that enhances and perpetuates the profibrotic effects of TGF-beta.

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.

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.

Clinical

History

  • Morphea is usually asymptomatic, and the development of lesions is typically insidious. One exception is the acute, painful onset of eosinophilic fasciitis.
  • Arthralgias, usually localized to an affected extremity, may be reported by patients with morphea. Linear and deep lesions can also be associated with arthritis, myalgias, carpal tunnel syndrome, and other peripheral neuropathies.
  • Patients with craniofacial linear morphea can present with seizures (typically complex partial), headaches, cranial nerve palsies, trigeminal neuralgia, hemiparesis/muscle weakness, eye pain, and visual changes.

Physical

Physical findings in morphea are localized to the affected skin and underlying tissues, with varying configurations (eg, oval, linear, ill defined) and depths of involvement in the subtypes. Although subdivision of morphea by subtype is useful with regard to differences in epidemiology, anatomic site, and course of disease, it is important to recognize that continuous clinical and histologic transitions exist among all the variants within the morphea spectrum.

Plaque-type morphea is the most common and benign morphea subtype and includes guttate and keloidal (nodular) variants (see Media File 1). These lesions are relatively superficial, primarily involving the dermis.

Generalized morphea is a more extensive and severe form of plaque-type disease.

Linear morphea includes the en coup de sabre and Parry-Romberg variants (see Media File 2). It often qualifies as deep morphea (albeit in a linear pattern), involving the deep dermis, subcutaneous fat, muscle, bone and even underlying meninges and brain.

Deep morphea, also referred to as subcutaneous morphea or morphea profunda, primarily involves the subcutaneous fat and underlying structures such as fascia (see Media File 4). Variants of deep morphea include eosinophilic fasciitis and disabling pansclerotic morphea of children.

  • Types of skin lesions
    • Plaque-type morphea lesions are characterized as circumscribed, indurated plaques that range from 1 cm to more than 20 cm in diameter. They often begin as erythematous to violaceous patches or slightly edematous plaques. With disease progression, sclerosis develops centrally as the lesions undergo peripheral expansion. The surface becomes smooth and shiny over time, with loss of hair follicles and sweat glands. The margins are often surrounded by a zone of violaceous color or telangiectasias. Over a period of months to years, the skin softens and the dermis becomes atrophic.
    • Guttate morphea lesions are small (<10 mm in diameter) and superficial, with less induration and a sharply demarcated border. The clinical appearance overlaps with that of lichen sclerosus, but true guttate morphea lacks epidermal atrophy and follicular plugging.
    • Keloidal (nodular) morphea is a rare variant characterized by nodules resembling keloids in the presence of typical plaque-type morphea.
    • Atrophoderma of Pasini and Pierini is thought to represent an abortive form of morphea and resembles "burnt-out" plaque-type lesions. It is characterized by hyperpigmented, slightly depressed areas with well-defined "cliff-drop" borders and no obvious induration. Similar hyperpigmented patches with minimal induration are seen in persons with superficial morphea, which, unlike atrophoderma of Pasini and Pierini, is characterized histologically by sclerosis of the upper dermis.
    • Bullous morphea is a rare variant in which tense subepidermal bullae develop overlying plaque-type, linear, or deep morphea lesions. This phenomenon may result from stasis of lymphatic fluid due to the sclerodermatous process or coexisting lichen sclerosus.
    • Linear morphea features discrete, indurated linear bands.
    • Frontoparietal linear morphea, called en coup de sabre, is characterized by a linear, atrophic depression suggestive of a stroke from a sword. Such lesions may extend deep into underlying tissues (see Media File 3).
    • Parry-Romberg syndrome (progressive hemifacial atrophy) is thought to represent a severe, segmental form of craniofacial linear morphea. Unlike en coup de sabre, the primary abnormality occurs in the subcutaneous fat, muscle, and bone. Although the skin is typically not indurated or bound down, some patients also exhibit primary cutaneous sclerosis reminiscent of en coup de sabre.
    • Deep morphea is characterized by ill-defined, bound-down, sclerotic plaques with a "cobblestone" or "pseudo-cellulite" appearance. The "groove sign" (a depression along the course of a vein, between muscle groups, or both) may be evident later in the course of disease.
    • Eosinophilic fasciitis (Shulman syndrome) involves primarily the fascia and is characterized by an acute onset of symmetric pain and edema of the extremities, followed by progressive induration with an appearance similar to deep morphea.
    • Disabling pansclerotic morphea of children has generalized involvement that extends throughout the tissues from dermis to bone.
  • Color of skin lesions
    • Plaque-type morphea often begins as a discrete area of erythema. With progression of sclerosis, the center of the lesion gradually develops a waxy, ivory color. In active phases of the disease, a violaceous border (lilac ring) may surround the indurated region. Hyperpigmentation often ensues as lesions evolve and eventually involute. The sclerotic lesions of guttate morphea are typically whitish in color, and the clinical appearance may overlap with that of extragenital lichen sclerosus. The patches of atrophoderma of Pasini and Pierini are hyperpigmented.
    • The multiple, coalescent lesions of generalized morphea are often hyperpigmented to silvery.
    • Deep morphea lesions are frequently hyperpigmented, but, because of the deeper level of inflammation, they lack the other color changes typical of plaque-type morphea.
  • Shape of skin lesions
    • Plaque-type morphea lesions are typically oval or round.
    • Linear morphea occurs as a linear band.
    • Deep morphea lesions tend to be ill defined.
  • Palpation of skin lesions
    • Well-developed morphea lesions are typically indurated and firm to palpation.
    • Linear and deep morphea lesions are often fixed to underlying structures, reflecting their extension down to muscle or bone. Older lesions may be either atrophic or sclerotic.
  • Arrangement of skin lesions
    • Patients can present with single or multiple plaque-type morphea lesions. Oval plaques on the trunk are often oriented with their long axes in a horizontal direction and typically have an asymmetric distribution. Guttate morphea lesions are multiple.
    • Linear morphea lesions are most often single and are unilateral in 95% of cases. If both the upper and lower extremities are involved, lesions are usually homolateral. Although a few cases of linear morphea following Blaschko lines have been described, most lesions do not obviously correspond to Blaschko lines. Linear morphea usually extends along the length of an extremity, but sometimes a band surrounds a limb or finger circumferentially, resembling ainhum (a constriction band that can lead to amputation of a digit).
    • Deep morphea is characterized by diffuse thickening of subcutaneous tissues with ill-defined borders. Distribution of lesions is often symmetric.
  • Areas of distribution
    • Plaque-type morphea is more common on the trunk (especially the lower aspect) than on the extremities, and the face is usually spared. Guttate morphea primarily involves the neck and the upper portion of the trunk. Atrophoderma of Pasini and Pierini usually occurs on the back, whereas superficial morphea favors intertriginous sites such as the axillae and inner thighs.
    • Generalized morphea occurs when morphea plaques become confluent or multiply and affect a significant portion of 3 or more major anatomical regions, often involving the chest, abdomen, lower back, buttocks, and thighs. In a rare variant of almost universal morphea, the whole body, from the top of the head to the feet, is involved; unlike diffuse systemic scleroderma, patients lack sclerodactyly, Raynaud phenomenon, or internal involvement.
    • Linear morphea most often occurs on the lower extremities, followed in frequency by the upper extremities, frontal portion of the head, and anterior trunk. En coup de sabre is the term used for linear morphea affecting the frontoparietal aspect of the face and scalp. Paramedian lesions are more common than median lesions.
    • Eosinophilic fasciitis most often affects the extremities, sparing the fingers and toes; the trunk is occasionally involved. Disabling pansclerotic morphea of children begins on the extensor extremities and progresses to the trunk, flexor extremities, face, and scalp, eventually sparing only the fingertips and toes.
  • Hair and nails
    • Scalp involvement results in scarring alopecia, as seen in en coup de sabre. Loss of eyebrows and eyelashes can also occur in this variant.
    • Nail dystrophy may develop when linear lesions involve the nail matrix and in pansclerotic morphea.
  • General examination
    • Extensive truncal morphea may lead to restricted respiration.
    • When linear or deep morphea lesions cross joint lines, they can cause restricted mobility, contractures, and deformity. In children, such lesions can result in growth impairment and severe atrophy of affected limbs.
    • Muscle weakness may occur in patients with central nervous system abnormalities related to craniofacial linear morphea and in those with peripheral nerve involvement by morphea on an extremity. Signs of carpal tunnel syndrome may be evident in patients with deep morphea affecting the wrist (especially eosinophilic fasciitis).
    • Ocular manifestations of craniofacial morphea include ptosis, extraocular muscle dysfunction, anterior uveitis, episcleritis, glaucoma, xerophthalmia, and keratitis.
    • Oral findings in patients with craniofacial morphea include altered dentition, malocclusion, and asymmetry of the tongue.

Causes

The cause of morphea is unknown. To date, investigations have not uncovered any consistent etiologic factors. Different morphea subtypes often coexist in the same patient, suggesting that the underlying processes are similar.

  • Radiation therapy: Morphea can occur at the site of previous supervoltage radiation therapy for breast cancer and other malignancies, developing from 1 month up to more than 20 years after irradiation.
  • Infection or vaccination
    • Infections, such as Epstein-Barr virus infection, varicella, measles, and borreliosis, have been reported to precede the onset of morphea and have been proposed as possible triggers.
    • The most extensive literature focuses on Borrelia burgdorferi as a possible etiologic agent for morphea. Some studies have detected Borrelia DNA within morphea lesions from a subset of European and Japanese patients (representing Borrelia afzelii and Borrelia garinii rather than B burgdorferi sensu stricto, the predominant subtype in the United States); however, to date, this has not been demonstrated in patients from the United States. Several more recent studies found no serologic or polymerase chain reaction–based evidence of Borrelia infection in patients with morphea.
    • Morphealike lesions have also been reported to occur after BCG and tetanus vaccinations.
  • Trauma: Some morphea patients report a history of local trauma directly preceding the onset of disease. Excessive physical exertion triggers eosinophilic fasciitis in approximately half the cases.
  • Genetics: A few familial cases of morphea have been reported, most commonly the disabling pansclerotic subtype. No significant HLA associations have been described.

More on Morphea

Overview: Morphea
Differential Diagnoses & Workup: Morphea
Treatment & Medication: Morphea
Follow-up: Morphea
Multimedia: Morphea
References
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Further Reading

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Keywords

localized scleroderma, plaque-type morphea, atrophoderma of Pasini and Pierini, guttate morphea, keloidal morphea, nodular morphea, bullous morphea, generalized morphea, linear morphea, linear scleroderma, en coup de sabre, progressive hemifacialatrophy, progressive facial hemiatrophy, Parry-Romberg syndrome, deep morphea, morphea profunda, subcutaneous morphea, eosinophilic fasciitis, Shulman syndrome, disabling pansclerotic morphea

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Contributor Information and Disclosures

Author

Kendra G Bergstrom, MD, Staff Physician, Ronald O Perelman Department of Dermatology, New York University School of Medicine
Kendra G Bergstrom, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Coauthor(s)

Julie V Schaffer, MD, Assistant Professor, Departments of Dermatology and Pediatrics, New York University School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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