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Morphea Workup

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

Laboratory Studies

Laboratory tests have a limited role in the evaluation of patients with morphea. The following studies can be considered on a case-by-case basis (eg, to monitor disease activity) but generally are not required:

CBC count

CBC count results are usually normal. Peripheral eosinophilia is most often present in patients with eosinophilic fasciitis and forms of deep morphea, but it may be observed in those with early, active morphea of any type.

Erythrocyte sedimentation rate

This is usually normal, but it may be elevated in patients with extensive, active morphea.

Autoantibodies

Serum autoantibodies are commonly present in all types of morphea. Their clinical and prognostic significance remains unclear.

Rheumatoid factor is positive in 15-60% of morphea patients, most often children with linear morphea.

Antinuclear antibodies are present in approximately 20-80% of morphea patients, typically with a homogeneous, speckled, or nucleolar pattern. The prevalence is higher in patients with generalized, linear, and deep subtypes.

Anti–single-stranded DNA antibodies are present in 25% of patients with plaque-type morphea, in 75% of those with generalized morphea, and in 50% of those with linear morphea; levels correlate with extensive, active disease and joint contractures.[35]

Antihistone antibodies are present in 47-87% of morphea patients overall and in 85% of those with generalized morphea, correlating with the number of plaque-type lesions and the total area affected.[36] The antihistone antibody titers may be related to the extent of involvement and the disease activity in linear scleroderma.[35]

Anticentromere, anti-Scl70, and anti–double-stranded DNA antibodies are present in less than 5% of morphea patients.[4]

Antibodies to matrix metalloproteinase (MMP)-1 have shown to be significantly elevated in 46% of morphea patients.[37]

Antiphospholipid antibodies are present in some morphea patients. Immunoglobulin M and immunoglobulin G anticardiolipin antibodies are present in 60% and 25% of patients with generalized morphea, respectively. Lupus anticoagulant can also be detected in approximately 50% of this subgroup of patients.

Antitopoisomerase II-alpha antibodies are present in 76% of morphea patients.

Anti-Cu/Zn-superoxide dismutase antibodies are present in 90% of morphea patients.

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Imaging Studies

Radiography

Radiography may be helpful in cases of linear (including en coup de sabre) or deep morphea in which involvement of the underlying bone is suspected. It can also be used to monitor pediatric patients for potential growth defects.

Magnetic resonance imaging

MRI of the brain and skull in patients with en coup de sabre and Parry-Romberg syndrome may reveal abnormalities such as cortical atrophy, subcortical calcifications, white matter lesions, ventricular dilatation, leptomeningeal enhancement, anomalous intracranial vasculature, and skull atrophy, even in the absence of neurological symptoms.[22]

MRI is useful for demonstrating the depth of lesions in deep and generalized morphea. Typical findings include diffuse edema of the subcutaneous tissues with thickening, increased signal intensity on T2-weighted images, and contrast enhancement of the fascial planes.

Ultrasonography

The 10- to 25-MHz ultrasound can measure skin thickness, which correlates with disease activity. The 10- to 15-MHz ultrasound is available in the United States. Ultrasonography can be of great benefit in the evaluation and monitoring of localized scleroderma.[38, 39] Color Doppler ultrasound has also been used to assess disease activity.[40]

Laser Doppler flowmetry and infrared thermography

These can be complementary tools to detect active lesions.[13]

Electroencephalography

Abnormalities may be observed in patients with craniofacial linear morphea, usually localized to areas of the brain underlying affected skin, and sometimes without a history of clinical seizure activity.

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Other Tests

Outcome measures currently used in morphea studies include durometer measurements of skin hardness, cutometer measurements of skin elasticity and relaxation, and computerized methods for assessing skin lesions.[41] Clinical skin scoring methods include the modified skin score, based on degree of thickening and percent involvement, as well as the Localized Scleroderma Skin Severity Index (LoSSI), Localized Scleroderma Skin Damage Index (LoSDI), and Physician Global Assessment of Localized Scleroderma disease Damage, which take into account disease activity and damage.[42]

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Procedures

Although a presumptive diagnosis of morphea can frequently be made based on clinical findings, a biopsy can be used to confirm the diagnosis and delineate the depth of involvement.

For superficial circumscribed and generalized morphea, a deep punch biopsy (including subcutaneous fat) is usually sufficient. Different histologic features are seen at the sclerotic center versus the inflammatory border of the lesion, and thus the location of the biopsy should be noted.

For linear and deep morphea, an incisional biopsy extending down to muscle is required to document fascial involvement.

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Histologic Findings

The histologic findings of morphea and systemic sclerosis are similar, with a fundamental process of thickening and homogenization of collagen bundles. The depth of involvement is important for categorization into the morphea subtypes. The sclerotic process in superficial circumscribed morphea is centered in the lower reticular dermis, whereas other variants are characterized by replacement of the subcutaneous fat and underlying tissues by collagen. See the image below.

Histopathology of mature scleroderma; full-thickne Histopathology of mature scleroderma; full-thickness sclerosis of the dermis. Photomicrograph courtesy of Dirk Elston, MD.

The epidermis is usually normal, but rete ridges may become flattened later in the disease course.

In the early inflammatory stage, a perivascular and interstitial variably dense infiltrate of lymphocytes admixed with plasma cells and occasional eosinophils is observed in the reticular dermis and/or the fibrous trabeculae of the subcutaneous tissues. Blood vessel walls demonstrate endothelial swelling and edema, and thickening of preexisting collagen bundles and deposition of fine, wavy fibers of newly formed collagen occur.

In the late sclerotic stage, the inflammatory infiltrate typically disappears. Collagen bundles in the reticular dermis and subcutis become thick, closely packed, and hyalinized. Atrophic eccrine glands appear to be trapped within the middle of the thickened dermis as subcutaneous fat is replaced by collagen. A paucity of blood vessels is seen, and adnexal structures are progressively lost. Depending on the subtype, the process of sclerosis may extend into the fascia and even underlying muscle; in contrast, thickened collagen bundles are restricted to the dermis in superficial morphea.

Reports of lichen-sclerosus–like changes (edematous, homogenized collagen) in the papillary dermis have been described in lesions of morphea.[43]

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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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