| Localized Scleroderma:
Morphea |
|
|
|
| Causes
of Morphea Scleroderma |
|
| 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. |
| From
circumscribed scleroderma (morphea) to subcutaneous panniculitis-like
T-cell lymphoma: case report. A 45-year-old male Croat
presented with a 6-month history of tender erythematous
subcutaneous skin lesions without systemic symptoms. Analysis
of a lesion biopsy specimen showed circumscribed scleroderma
(morphea). Pathohistologic analysis and immunohistochemistry
of a specimen obtained by tumor excision from the back
indicated subcutaneous panniculitis-like T-cell lymphoma.
This case report illustrates the importance of continuous
follow-up with repeat biopsy and use of immunohistologic
techniques for early diagnosis. PubMed.
Acta Dermatovenerol Croat. 2004;12(4):289-93. (Also see: Scleroderma
and Cancer) |
| Guttate
morphoea in human T-cell lymphoma/lymphotrophic virus type-1
(HTLV-1) infection. A 62-year-old Japanese man presented
with multiple small atrophic macules on the trunk and extremities.
The clinico-pathological features were consistent with guttate
morphoea, a rare variant of localized scleroderma. PubMed.
Clin Exp Dermatol. 2003 Jul;28(4):380-2. |
| 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) |
| Linear
scleroderma along blaschko's lines in a patient with systematized
morphea. PubMed. Acta Derm
Venereol. 2003;83(5):362-4. (Also see: Linear
Scleroderma ) |
| 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. |
| IgG
antibody reactivity to Borrelia burgdorferi sensu stricto
antigens in patients with morphea in Colombia. This
does not rule out the possibility that a new spirochetal
agent, unrelated to B. burgdorferi or Treponemas, may be
the causative agent of morphea/LSA in Colombia. PubMed.
Int J Dermatol. 2003 Nov;42(11):882-6. |
| Morphoea Is Neither Associated
With Features of Borrelia Burgdorferi Infection, Nor Is This
Agent Detectable in Lesional Skin by Polymerase Chain Reaction "In
1985, Aberer and colleagues submitted a letter to the Lancet
proposing a causative link between spirochetal infection
and morphea,[1] and since then the topic has been highly
controversial. During the past decade and a half, evidence
has accumulated both in support of and against the association.[2-5]
Weide and colleagues now provide compelling data that may
finally settle the controversy about "Borrelia-induced
morphea." They conclude that there is no association." British
Journal of Dermatology October 2000. (Volume 142, Number
4) |
| 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. |
| Circumscribed
scleroderma belongs to the groups of the diseases characterized
by fibrosis of the skin and the deeper tissue. According
to the published data, 47% to 76% patients with circumscribed
scleroderma present antinuclear antibodies (ANA) as well
as antibodies to Borrelia burgdorferi which is believed to
be causative factor of the disease. The study was conducted
in the group of 50 consecutive patients with circumscribed
sclerosis (en plaque, deep linear, atrophoderma Pasini-Pierini).
The role of the ANA with no distinctive specificity and antibodies
to Borrelia burgdorferi in the localized scleroderma is still
unclear. PubMed. Przegl Lek 2002;59(11):898-902. |
| 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 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) |
| Localized
scleroderma in a woman irradiated at two sites for endometrial
and breast carcinoma: A case history and a review of the
literature. Localized scleroderma is an uncommon side-effect
of radiotherapy. We report a unique case with multiple asynchronous
primary malignant tumors, which developed localized scleroderma
after radiotherapy. PubMed. Int
J Gynecol Cancer 2003 Jan-Feb;13(1):77-82 (Also see: Causes
of Scleroderma: Radiation and Localized
Scleroderma) |
| 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) |
| Tumor Necrosis Factor
(TNF) |
| TNF and IL-13: Serum levels of tumor necrosis factor and interleukin-13
are elevated in patients with localized scleroderma. These
results suggest that TNF and IL-13 may be associated with
the development of LSc. PubMed.
Dermatology. 2003;207(2):141-7. (Also see: Linear and Antibodies) |
| Vitamin B12 |
| Vitamin
B12-associated localized scleroderma and its treatment. We
report the first non-European case of localized sclerodermoid
reaction secondary to vitamin B12 injections. The patient
was successfully treated with excision of diseased skin,
local fat transfer, and complex repair with dramatic improvement
at 5 months in contour and overall appearance. PubMed.
Dermatol Surg. 2004 Sep;30(9):1252-5. |
