Morphea Clinical Presentation

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

History

Morphea is usually asymptomatic, and the development of lesions is typically insidious. One exception is the acute, painful onset of eosinophilic fasciitis.

Extracutaneous involvement is present in 20% of patients.[10] Extracutaneous manifestations are more common in the linear and generalized subtype.

  • 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.
  • Dysphagia (esophageal dysmotility or reflux), dyspnea, and vascular complaints also are reported.
  • 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 secondary to involvement of the underlying central nervous system.
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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

Plaque-type morphea, as shown in the image below, is the most common and benign morphea subtype.

Inflammatory plaque-type morphea on the abdomen, cInflammatory plaque-type morphea on the abdomen, characterized by induration, erythema, and a surrounding lilac ring.

Plaque-type lesions are characterized as circumscribed, indurated plaques that range from 1 cm to more than 20 cm in diameter. These lesions are relatively superficial, primarily involving the dermis. They often begin as oval-round erythematous to violaceous patches or slightly edematous plaques. In active phases of the disease, a violaceous border (lilac ring) may surround the indurated region. With disease progression, sclerosis develops centrally as the lesions undergo peripheral expansion. Over a period of months to years, the surface becomes smooth, shiny, and ivory in color over time, with loss of hair follicles and sweat glands. Hyperpigmentation often ensues as lesions evolve and eventually involute.

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. Plaque-type morphea is more common on the trunk (especially the lower aspect) than on the extremities, and the face is usually spared.

Variants of plaque-type morphea are as follows:

  • Guttate morphea lesions are multiple and primarily involve the neck and the upper portion of the trunk. The lesions are small (< 10 mm in diameter) and superficial, with less induration and a sharply demarcated border. The sclerotic lesions of guttate morphea are typically whitish in color. The clinical appearance may overlap with that of extragenital 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 typically located on the trunk and 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. Superficial morphea favors intertriginous sites such as the axillae and inner thighs.
  • 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.

Generalized morphea

Generalized morphea is a more extensive and severe form of plaque-type disease. 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. The multiple, coalescent lesions of generalized morphea often range from hyperpigmented to silvery. 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, nailfold capillary changes, or internal involvement.

Linear morphea

Linear morphea, as shown in the image below, includes the en coup de sabre and Parry-Romberg variants

A hyperpigmented band of linear morphea involving A hyperpigmented band of linear morphea involving the right part of the trunk and thigh.

Linear morphea often qualifies as deep morphea (albeit in a linear pattern), involving the deep dermis, subcutaneous fat, muscle, bone, and even underlying meninges and brain. Linear morphea features discrete, indurated linear bands that are most often single and are unilateral in 95% of cases. Older lesions may be either atrophic or sclerotic.

Linear morphea most often occurs on the lower extremities, followed in frequency by the upper extremities, frontal portion of the head, and anterior trunk. Many cases of linear morphea following Blaschko lines have been described, although most lesions do not obviously correspond to Blaschko lines.[11, 12] 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). Nail dystrophy may develop when linear lesions involve the nail matrix and in pansclerotic morphea.

Frontoparietal linear morphea, called en coup de sabre, is characterized by a linear, atrophic depression affecting the frontoparietal aspect of the face and scalp, suggestive of a stroke from a sword, as shown in the image below. Paramedian lesions are more common than median lesions. Such lesions may extend deep into underlying tissues. Scalp involvement results in scarring alopecia. Loss of eyebrows and eyelashes can also occur in this variant.

Linear atrophic depression of an en coup de sabre Linear atrophic depression of an en coup de sabre lesion on the right side of the forehead and the frontal part of the scalp.

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

Deep morphea, as seen in the image below, is also referred to as subcutaneous morphea or morphea profunda and primarily involves the subcutaneous fat and underlying structures such as fascia.

Morphea profunda involving the left lower extremitMorphea profunda involving the left lower extremity, with thickened, taut, bound-down skin.

Variants of deep morphea include eosinophilic fasciitis and disabling pansclerotic morphea of children.

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. Distribution of lesions is often symmetric. 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.

Eosinophilic fasciitis (Shulman syndrome) involves primarily the fascia and is characterized by an acute onset of symmetric pain and edema of the extremities or trunk, followed by progressive induration with an appearance similar to deep morphea. Eosinophilic fasciitis most often affects the extremities, sparing the fingers and toes; the trunk is occasionally involved.

Disabling pansclerotic morphea of children has generalized involvement that extends throughout the tissues from dermis to bone. It begins on the extensor extremities and progresses to the trunk, flexor extremities, face, and scalp, eventually sparing only the fingertips and toes.

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.[13] 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.
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Causes

The cause of morphea is unknown. An autoimmune mechanism is suggested by an increased frequency of autoantibody formation and a higher prevalence of personal and familial autoimmune disease in affected patients.[14] The generalized subtype has a higher association with autoimmunity, with a higher frequency of concomitant autoimmune disease, systemic findings, and positive antinuclear antibody findings.[3] To date, investigations have not described 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.[15]
  • Chimerism: Immature chimeric cells have been found in morphea lesions, suggesting that such nonself cells may lead to an autoimmune phenotype.[10]
  • Infection or vaccination
    • Infections, such as Epstein-Barr virus infection, varicella, measles, hepatitis B, 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). Studies have shown an increased frequency of B burgdorferi in active morphea lesions by immunohistochemistry[16] ; however, to date, this has not been demonstrated in patients from the United States.[17, 18]
    • Antibodies to B burgdorferi and high antinuclear antibody titers have been shown to be associated with early-onset morphea.[14] Thus, Borrelia -associated early-onset morphea may represent a subset of patients with infection-induced autoimmunity. However, this subset of patients is not reflective of patients with morphea as a whole, because subsequent studies have not found serologic or polymerase chain reaction–based evidence of Borrelia infection in patients with morphea.[19, 20]
    • Morphealike lesions have also been reported to occur after BCG, tetanus, and mumps-measles-rubella vaccinations. Whether the vaccinations themselves or the trauma from the injections was the inciting event is not clear.
  • Drug-induced morphea: This is only rarely reported (ie, from bleomycin, D-penicillamine, L-5-hydroxytryptophane, balicatib).[21]
  • 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.
  • Hormones
  • Genetics: A few familial cases of morphea have been reported, most commonly the disabling pansclerotic subtype. No significant HLA associations have been described.
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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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