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Localized Scleroderma: Morphea
Overview
Types of Morphea
Juvenile Scleroderma
Associated Conditions
Causes of Morphea
Complications of Morphea
Diagnosis
Photos
Research Registry
Treatments
Patient and Caregiver Stories
Causes of Morphea Scleroderma
Autoimmunity
Cancer, Lymphoma
Dendritic Cells
Genetics
Infections
Borrelia Burgorferi
HTLV-1 Infection
Toxoplasma Gondii
Medications
Valproic Acid
Radiation
Solvents, Organic Solvents
Overview of Causes of Morphea Scleroderma
The cause of morphea scleroderma is unknown in most cases, however sometimes it has been association with cancer, infections, medications, radiation, solvents, and vitamin B12. Gene profiling is underway to determine the genes associated with the development of morphea.
Morphea is a form of localized scleroderma, which usually affects only the skin or underlying muscles and joints.

Generally speaking, localized scleroderma is substantially different from systemic scleroderma, since systemic scleroderma often affects the skin, blood vessels, and/or internal organs. (Also see: Morphea Scleroderma, Types of Scleroderma and Causes of Systemic Scleroderma)

Autoimmunity
Localized scleroderma is an autoimmune disorder. Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis. Rheumatology (Oxford). 2005 Mar;44(3):274-9. Epub 2004 Nov 23. (Also see: ISN Medical Advisory Board: Dr. Shinichi Sato)
Cancer, Lymphoma, HTLV-1 Infection
Morphea has been associated with T-cell lymphoma in several case reports. A very rare form of T-cell lymphoma is due to a virus (an HTLV-1 infection) and this virus has been associated in at least one case with guttate morphea. When this type of lymphoma is identified by high calcium in blood, bone and skin lesions, and lymphoma cells in blood and bone marrow, your doctor will order special blood tests to check for the virus.
Scleroderma and Cancer. Systemic scleroderma may be associated with an increased incidence of cancer, including breast cancer, B-cell lymphoma, lung cancer and tongue cancer. ISN.
Dendritic Cells
Immunophenotyping of chimeric cells in localized scleroderma. We report that not only are chimeric cells present in affected localized scleroderma lesions but they also are more likely to be dendritic cells and B lymphocytes suggesting a role in the pathogenesis of localized scleroderma. PubMed. Rheumatology (Oxford). 2006 Nov 4. (Also see: Fetal Cells and Scleroderma)
Genetics
Gene expression studies in Scleroderma and Morphea. Initial examination of our data suggests that some differences between unaffected and affected areas of skin do exist, although they are not as great as the differences between volunteer control skin samples and the patient samples. Scleroderma Assoc. of NSW. Report from Professor K Stanley, Scleroderma Laboratory, St Vincent's Hospital, NSW.
Gene profiling to start with Morphea In the last quarter we have identified genes in all of the major skin cell types that can be used as reference points for gene expression. We have also identified genes that are highly activated in each cell type in response to inflammation. This will allow us to determine which cell types are becoming activated. Scleroderma Assoc. of NSW. August 2003. (Also see: Causes of Scleroderma: Genetics)
Infections
Borrelia burgdorferi
Morphoea: a manifestation of infection with Borrelia species? The frequent detection of this microorganism in morphoea points to a specific involvement of B. burgdorferi or other similar strains in the development of or as a trigger of this disease. PubMed. Br J Dermatol. 2007 Oct 17.
Dermatological aspects of Lyme borreliosis. The relationship between infection with B. burgdorferi and other dermatoses, especially morphea, lichen sclerosus, and interstitial granulomatous dermatitis is still debated. (PubMed) Med Mal Infect. 2007 Jul-Aug;37(7-8):540-7.
HTLV-1 Infection, Lymphoma, and Morphea
Causes of Morphea: Cancer. Morphea has been associated with T-cell lymphoma in several case reports. See above, Causes of Morphea: Cancer.
Toxoplasma gondii
Circumscribed scleroderma or morphea is a rare disease that involves limited areas of skin and usually is not associated with visceral lesions. We present a case of a male patient, which developed morphea lesions during the infection with Toxoplasma gondii. The evolution of lesions was correlated with antibodies titre for Toxoplasma gondii, and local administration of corticosteroids accelerated the evolution to cutaneous lesions stabilization. PubMed. Rev Med Chir Soc Med Nat Iasi. 2003 Jul-Sep;107(3):646-9.
Medications
Valproic Acid
Localized morphea: a rare adverse effect of valproic acid. To our knowledge, this is the first patient reported to have an apparently valproic acid-induced localized morphea. PubMed. Pediatr Neurol. 2003 Sep;29(3):253-5.
Radiation
Postirradiation morphea in a breast cancer patient. It is important to be aware of this rare complication of radiation therapy because it clinically presents with symptoms mimicking malignancy and may be misinterpreted as recurrent carcinoma or even angiosarcoma. PubMed. Breast J. 2006 Mar-Apr;12(2):173-6. (Also see: Cancer and Scleroderma)
Postirradiation morphea and subcutaneous polyarteritis nodosa: Case report and literature review. Postirradiation morphea is an uncommon condition but is being increasingly recognized. Related phenomena following radiation include postirradiation panniculitis and now postirradiation subcutaneous polyarteritis nodosa. Radiation may be responsible for inducing some of the pathogenic changes seen in scleroderma and other autoimmune diseases. PubMed. Semin Arthritis Rheum. 2005 Apr;34(5):728-34. (Also see: Associated Conditions: PAN, and Causes of Scleroderma: Radiation)
Solvents, Organic Solvents
Amyloid deposition associated with generalized morphea-like scleroderma. (The patient) had been occupationally exposed to organic solvents. As far as we know, amyloid deposition associated with generalized morphea-like scleroderma has not been reported until now. PubMed. Eur J Dermatol. 2003 Sep-Oct;13(5):509-11. (Also see: Causes of Scleroderma: Environmental)
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