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Morphea Clinical Presentation

  • Author: Jennifer V Nguyen, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Nov 18, 2015
 

History

Morphea is usually asymptomatic, and the development of lesions is typically insidious.

Extracutaneous involvement is present in 20% of patients.[13] Extracutaneous manifestations are more common in the linear and generalized subtypes.

Malaise, fatigue, myalgias, and arthralgias are common extracutaneous symptoms. Arthralgias are usually localized to an affected extremity. Linear and deep lesions can also be associated with arthritis, carpal tunnel syndrome, and other peripheral neuropathies.

Dysphagia (esophageal dysmotility or reflux), dyspnea, and vascular complaints also are reported.

Neurologic manifestations, which are more common in patients with en coup de sabre or progressive hemifacial atrophy, include 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.

Dry eyes are also frequently reported due to eyelid or lacrimal gland sclerosis.

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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.

Circumscribed morphea

Circumscribed morphea, also known as morphea en plaque, is the most common subtype of morphea. Patients present with fewer than 3 discrete lesions, predominantly on the trunk. Circumscribed morphea may be divided into superficial and deep variants, with the superficial variant being the most common.

Superficial variant

In the superficial variant, 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. See the image below.

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

Patients can present with single or multiple lesions. Oval plaques on the trunk are often oriented with their long axes in a horizontal direction and typically have an asymmetric distribution. Superficial circumscribed morphea is more common on the trunk (especially the lower aspect) than on the extremities, and the face is usually spared. Plaques often develop in areas of pressure, such as the hips, around the waist, and around the bra line in women.

Deep variant

The deep variant of circumscribed morphea (previously referred to as subcutaneous morphea or morphea profunda) primarily involves the subcutaneous fat and underlying structures such as muscle and fascia. 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. The overlying epidermis may be uninvolved, atrophic, or indurated. The lesions are frequently hyperpigmented, but because of the deeper level of inflammation, they lack the other color changes typical of superficial circumscribed morphea. Distribution of lesions is often symmetric. See the image below.

Deep morphea involving the left lower extremity, w Deep morphea involving the left lower extremity, with thickened, taut, bound-down skin.

Other variants of circumscribed morphea

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 a superficial 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.

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, occurring in 7-9% of morphea patients. Generalized morphea is defined as more than 4 indurated plaques larger than 3 cm each and/or involving 2 of more of 7 anatomic sites (head-neck, each extremity, anterior trunk, and posterior trunk) but sparing the face and hands. 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 trunk/limb, en coup de sabre, and Parry-Romberg variants.

A hyperpigmented band of linear morphea involving A hyperpigmented band of linear morphea involving the left 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 75-95% of cases.[14, 15] 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.[16, 17] 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 and may be associated with underlying ocular and central nervous system involvement. 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.

Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, 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, approximately 71% of patients also exhibit cutaneous sclerosis reminiscent of en coup de sabre.[18]

Pansclerotic morphea

Pansclerotic morphea of children is the most debilitating form of morphea. It 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. Significant morbidity, including muscle atrophy, joint contractures, and nonhealing ulcers, is associated.

Mixed

Mixed variant morphea refers to patients who exhibit 2 or more subtypes described above. It occurs in up to 15% of patients with morphea.[4, 15]

General examination

Extensive truncal morphea may lead to restricted respiration.

Complete physical examination is recommended, including genital and oral mucosa. Genital lichen sclerosus has been reported with high frequency in patients with morphea.[19, 20]

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, limb-length discrepancies, and severe muscle 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.[21] Signs of carpal tunnel syndrome may be evident in patients with deep morphea affecting the wrist.

Ocular manifestations are most common in the en coup de sabre variant of morphea and include ptosis, ectropion, extraocular muscle dysfunction, anterior uveitis, episcleritis, glaucoma, xerophthalmia, and keratitis.[22]

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.[4, 23] Patients with generalized morphea are more likely to have a concomitant autoimmune disease, positive serology for autoantibodies, particularly antinuclear antibody, and systemic symptoms.[4] 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. Note the following causes and associations:

  • Radiation therapy: Morphea can occur at the site of previous radiation therapy for breast cancer and other malignancies, developing from 1 month up to more than 20 years after irradiation. [24, 25] Involvement may extend beyond or distant to the irradiation field. [12]
  • Chimerism: Immature chimeric cells have been found in morphea lesions, suggesting that such nonself cells may lead to an autoimmune phenotype. [13]
  • 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[26] ; however, to date, this has not been demonstrated in patients from the United States.[27, 28]
    • Antibodies to B burgdorferi and high antinuclear antibody titers have been described in patients with morphea,[23] and it has been suggested that Borrelia -associated early-onset morphea may represent a subset of patients with infection-induced autoimmunity. However, results have been conflicting, as other studies have not found a definitive association between Borrelia infection and morphea based on serologic or polymerase chain reaction data.[29, 30, 31]
    • Morphealike lesions have also been reported to occur following vaccinations, including 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 bisoprolol, bleomycin, D-penicillamine, L-5-hydroxytryptophane, balicatib). [32, 12]
  • Trauma: Some morphea patients report a history of local trauma directly preceding the onset of disease. Plaques of circumscribed morphea often develop in areas of pressure. Reports of morphea lesions following vitamin B-12 and vitamin K injections suggests that trauma from injections may play a role. [12]
  • Hormones
  • Genetics: A few familial cases of morphea have been reported, most commonly the disabling pansclerotic subtype. No significant HLA associations have been described.
  • Chemical exposure (link) [33]
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Contributor Information and Disclosures
Author

Jennifer V Nguyen, MD Assistant Professor of Dermatology, 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, Phi Beta Kappa, Society for Investigative Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Nicole Fett, MD Assistant Professor, 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, Women's Dermatologic Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Acknowledgements

Kendra G Bergstrom, MD Staff Physician, Ronald O Perelman Department of Dermatology, New York University School of Medicine

Kendra G Bergstrom 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.

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 Girardi, MD Program Director, Assistant Professor, Department of Dermatology, Yale University School of Medicine

Disclosure: Nothing to disclose.

Julie V Schaffer, MD Assistant Professor, Departments of Dermatology and Pediatrics, New York University School of Medicine

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 left 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.
Deep morphea 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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