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Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Clinical Presentation

  • Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 16, 2015
 

History

The clinical presentation of morphea varies depending on the level of tissue involvement and extent of the lesions. The diagnosis is based more on physical examination than on history. Certain features, including localization of the lesions, help the clinician increase the index of suspicion for a specific diagnosis.

  • A common feature of morphea is skin thickening.
  • Internal organ involvement is not evident in most patients with morphea.
  • Some patients present with associated arthralgias, foot and hand deformities, central nervous system abnormalities, intermittent abdominal pain, and periosteal erosions and osteolysis.
  • Spina bifida is found in almost 33% of patients with morphea.
  • Associated cutaneous pathologies include alopecia areata, vitiligo, dystrophy of the nails, and ichthyosis.
  • Occasionally, localized morpheic lesions occur in the uninvolved skin of patients with progressive systemic sclerosis.
  • Morphea has been reported in association with systemic lupus erythematosus, primary biliary cirrhosis, pemphigus, and dermatomyositis.
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Physical

Plaque morphea

Superficial morphea are often confined to the dermis, as depicted in the images below, and occasionally to the superficial panniculus. Subsets include morphea en plaque, guttate morphea, keloid morphea, lichen sclerosus et atrophicus, and atrophoderma of Pasini and Pierini.

This photograph shows morphea en plaque on the tru This photograph shows morphea en plaque on the trunk of a patient. There is a distinctive border separating the plaque from the surrounding normal skin (reproduced with permission of Mayo Clinic Proceedings).
This photograph shows generalized morphea on the t This photograph shows generalized morphea on the trunk of a patient (reproduced with permission from Mayo Clinic Proceedings).

Morphea en plaque

This is the most prevalent form of this group. It involves only 1 or 2 anatomic sites, usually the trunk. The disease is characterized by one or more circular areas of induration, usually larger than 1 cm in diameter, with varying degrees of pigment changes.

An erythematous or violaceous halo (lilac ring), which is often evident during early disease, corresponds with an inflammatory state. As the disease progresses, the skin becomes sclerotic. As the inflammation subsides, the center of the lesion becomes whitish. After months to years, the skin softens and becomes atrophic, and a residual area of hypopigmentation or hyperpigmentation might occur. Distinctive borders usually separate the plaques from the normal surrounding skin.

Guttate morphea

This subset usually occurs on the upper trunk. Typically, it presents as multiple oval lesions between 2-10 mm in diameter. The lesions often manifest as faint erythema, followed by mild induration and hypopigmentation or hyperpigmentation.

Keloid morphea

This presents as nodules that resemble keloids in the presence of typical morphea.

Lichen sclerosus et atrophicus

This is characterized by shiny white plaques often preceded by violaceous discoloration of the skin. This subset has a predilection for the anogenital area and has an increased association with autoimmune-related diseases (eg, vitiligo, alopecia areata).

Atrophoderma of Pasini and Pierini

This presents as asymptomatic, hyperpigmented atrophic patches with well-demarcated "cliff drop borders" on the trunk. No pronounced inflammatory or sclerotic changes are present. The course is chronic, with spontaneous resolution usually after 10 years.

Generalized morphea

This occurs when individual plaques of morphea become confluent lesions. It can affect more than two anatomic sites, including the upper trunk, breasts, abdomen, and upper thighs. Arms, legs, face, neck, and scalp may also be involved.

Bullae may develop in localized areas, particularly around the abdomen. Keratoses and calcinosis may occur. Contractures may occur in limbs, and mobility may be restricted.

Bullous morphea

This is characterized by tense subepidermal bullae in the presence of typical morphea or morphea profunda. The lesions can occur on the face, neck, trunk, or extremities and may be superficial or extend deep into the dermis.

Linear scleroderma

This is characterized by one or more linear streaks and induration that can involve dermis, subcutaneous tissue, muscle, and bone. It occurs on the extremities, face, or scalp of children and adolescents. It is subdivided into subgroups including linear scleroderma, lesions en coup de sabre, and progressive hemifacial atrophy (Parry-Romberg syndrome).

Linear scleroderma

This is characterized by discrete linear induration that primarily affects the extremities. In more than 90% of patients, the involvement is unilateral. Linear scleroderma is complicated by deformities, joint contractures, and severe limb atrophy. The process can affect the growth of bony structures.

Lesions en coup de sabre

If the face or scalp is affected with linear scleroderma, the involvement is often compared to a stroke from a sword (sabre). The lesions start with contractions and firmness of the skin over the affected area. Then, an ivory, irregular, sclerotic plaque with hyperpigmentation at the edge develops.

Progressive hemifacial atrophy (Parry-Romberg syndrome)

This subtype results in hemiatrophy of the face. The primary lesions occur in the subcutaneous tissue, muscle, and bone. The disease usually begins in the first 2 decades of life.

Deep morphea

This primarily involves the deep dermis, subcutaneous tissue, fascia, or superficial muscle. The lesions are more diffuse than in linear scleroderma and do not demonstrate a linear pattern. Subtypes include subcutaneous morphea, morphea profunda, disabling pansclerotic morphea of childhood, and eosinophilic fasciitis.

Subcutaneous morphea

It primarily involves the panniculus or subcutaneous tissue. The onset of sclerosis is usually rapid, occurring during a period of several months. This is a more of an inflammatory condition than other types of morphea.

Morphea profunda

The entire skin feels thickened, bound down, and taut. Patients develop deep sclerosis of the skin. Onset usually occurs before age 14 years. The extensor aspect of the extremities and trunk develops sclerotic plaques that extend deep into the subcutaneous tissue, fascia, muscle, and bone. The lilac ring is usually absent.

Disabling pansclerotic morphea of childhood

This is an aggressive and mutilating variant of morphea. It is characterized by generalized, full-thickness involvement of the trunk, extremities, face, and scalp. The fingertips and toes are usually spared.

Eosinophilic fasciitis

This condition is characterized by a painful peau d'orange appearance involving the extremities, proximal to the hands and feet. The fascia is the predominant level of involvement. The natural course is not well defined, but patients seem to spontaneously regress or remain unchanged for years.

Regional fibrosing disorders

Mediastinal fibrosis is characterized by excessive perinodal fibrotic proliferation that invades and destroys normal mediastinal structures. It can coexist with retroperitoneal fibrosis. It is considered the most serious late complication of remote Histoplasma capsulatum infection. Tuberculosis has been considered another triggering agent. Mediastinal hemorrhage and methysergide use also can trigger mediastinal fibrosis.

Patients with mediastinal fibrosis can present with pleuritic chest pain, malaise, and pain and stiffness in the thoracic spine. Other symptoms include dyspnea, cough, hemoptysis, and superior vena caval obstruction. The fibrotic process may accrue over prolonged periods and may extend within the lumina of critical mediastinal structures to produce complete occlusion. Patients may develop severe stenosis of the trachea, carina, or mainstem bronchi.

An accentuated pulmonic component of the second heart sound, wheezing, and localized murmur are among the most common physical findings. Thoracotomy may be required for histologic diagnosis. Successful superior vena cava bypass surgery has been described. Corticosteroid therapy has been effective in some reported cases.

Retroperitoneal fibrosis, or Ormond disease, is a rare inflammatory fibrosing disorder that leads to compression of the ureter, vena cava, nerves, and aorta. The disorder is about twice as common in males as in females, and the peak age at onset is in the fifth and sixth decades of life. The course is usually slowly progressive in nature.

Low back pain is the most common symptom. Weight loss and fever are common. The most common clinical presentation of retroperitoneal fibrosis is renal dysfunction due to obstruction of the ureters. The retroperitoneal involvement may spread to the deep structures in the pelvis. Patients with retroperitoneal fibrosis can present with upper gastrointestinal bleeding and ascites due to complications of portal hypertension. Some patients may present with signs and symptoms of pulmonary hypertension associated with pulmonary arterial and venous obstruction.

Retroperitoneal fibrosis has been associated with the use of certain medications, including methysergide, ergotamine, pergolide (withdrawn from US market March 29, 2007), hydralazine, and methyldopa. Associated diseases include atherosclerotic disease of the aorta (abdominal aortic aneurysm), systemic lupus erythematosus, vasculitis, scleroderma, biliary cirrhosis, and malignancy.

Laboratory abnormalities are nonspecific, including an elevated erythrocyte sedimentation rate. The diagnosis is often suggested by findings of intravenous pyelography. Ultrasonography, CT scanning, and MRI are useful, showing a fibrotic paraaortic mass, as depicted in the image below. Gallium scintigraphy may help assess activity of inflammation. Multiple deep biopsies may be required to distinguish this condition from malignancy.

CT scan of the abdomen showing the typical paraaor CT scan of the abdomen showing the typical paraaortic mass of retroperitoneal fibrosis.

Surgical treatment is often highly successful. Steroid therapy may be useful in the cases detected before significant obstruction has occurred. Azathioprine, cyclophosphamide, and tamoxifen have been used as well, with apparent success. The long-term outlook is fairly good if the disease is recognized early before irreversible obstructive lesions have occurred. Most deaths are secondary to renal failure.

Dupuytren contracture consists of a thickening and shortening of the palmar fascia. Thick, cordlike superficial fibrous tissue, which can be felt in the palm, causes a contracture, usually of the fourth finger. The fifth, third, and second fingers are involved in decreasing order of frequency. Initial lesions might show dimpling or puckering of the skin over the involved fascia. Dupuytren contracture occurs predominantly in whites and is more common in Europe. The condition is 5 times more common in men. A gradual increase in incidence occurs with age. An association with diabetes and liver disease has been reported.

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Causes

See the list below:

  • The causes of localized fibrosing disorders are unknown.
  • Suggested causes include genetic, infectious, and autoimmune mechanisms. Neoplasms, infections, trauma, aortic aneurysms, and ergot have been associated with retroperitoneal fibrosis.
    • Autoantibodies are frequently seen in localized scleroderma, particularly antinuclear antibodies and antibodies to single-stranded DNA and histones.
    • Environmental agents (eg, L-tryptophan, vinyl chloride, bleomycin, methysergide, ergot, bromocriptine, radiation) can cause a morphealike picture. Methysergide is also associated with the development of retroperitoneal fibrosis. Other drugs that can trigger this condition include phenacetin, hydralazine, and propranolol.
    • Infections (eg, varicella, measles, Epstein-Barr virus, borreliosis) may precede the onset of morphea. H capsulatum infection has been associated with the development of mediastinal fibrosis.
    • Vaccination (bacille Calmette-Guérin [BCG]) has also been reported to precede the onset of morphea, and trauma has been considered a possible triggering event in these conditions.
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Contributor Information and Disclosures
Author

Mariana J Kaplan, MD Assistant Professor, Department of Internal Medicine, Division of Rheumatology, University of Michigan Medical School

Mariana J Kaplan, MD is a member of the following medical societies: American Association of Immunologists, American Federation for Medical Research, Central Society for Clinical and Translational Research, Clinical Immunology Society, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

References
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  9. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true connective tissue disease. Rheum Dis Clin North Am. 1996 Feb. 22(1):23-38. [Medline].

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  11. Kerscher M, Volkenandt M, Gruss C, et al. Low-dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. 1998 Jan. 38(1):21-6. [Medline].

  12. Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal fibrosis and histoplasmosis. Ann Thorac Surg. 1992 Dec. 54(6):1053-7; discussion 1057-8. [Medline].

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This photograph shows morphea en plaque on the trunk of a patient. There is a distinctive border separating the plaque from the surrounding normal skin (reproduced with permission of Mayo Clinic Proceedings).
This photograph shows generalized morphea on the trunk of a patient (reproduced with permission from Mayo Clinic Proceedings).
CT scan of the abdomen showing the typical paraaortic mass of retroperitoneal fibrosis.
 
 
 
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