| Localized Scleroderma:
Morphea |
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| What
is Morphea? |
The
most important thing to know about morphea is
that it is very different from the systemic
forms of scleroderma, and it is never fatal. |
 Morphea
scleroderma and its variants are also known as atrophoderma
of Pasini and Pierini, bullous
morphea, circumscribed scleroderma, deep morphea, disabling
pansclerotic morphea, en
coup de sabre, eosinophilic
fasciitis, generalized
morphea, guttate
morphea, keloid,
keloidal, or keloid-like morphea, linear
morphea, localized
scleroderma, morphea
profunda, nodular
morphea, pansclerotic
morphea, plaque-type morphea, progressive
hemifacial atrophy, progressive
facial atrophy, Parry
Rombergs, Shulman's
syndrome, and subcutaneous morphea. |
| When any form of scleroderma begins during childhood, it is
called juvenile
scleroderma. (Also see: Types
of Scleroderma) |
| Morphea scleroderma usually begins
as patches of yellowish or ivory-colored rigid, dry skin. These
then become hard, slightly depressed, oval-shaped plaques which
usually have a whitish or yellowish center surrounded by a
pinkish or purplish halo. |
| Morphea is most commonly found
on the trunk, but can occur anywhere on the body. About 75%
of the time it affects only the skin or underlying muscles
and joints. |
| Morphea Includes
background, pathophysiology, frequency, mortality/morbidity,
physical findings, causes, and treatments. emedicine dermatology. |
| Morphea,
also known as localized scleroderma, is a disorder characterized
by thickening and induration of the skin and subcutaneous tissue
due to excessive collagen deposition. Morphea subtypes are
classified according to their clinical presentation and depth
of tissue involvement; they include plaque-type, generalized,
linear, and deep varieties. eMedicine 2003. |
| Complications of Morphea |
| Plaque-type
scleroderma associated with linear and oesophageal features and
facial and extra-facial hemiatrophy. The combination of several
sub-types of scleroderma and facial and extra-facial hemiatrophy
in the same patient may indicate that these entities actually
represent different spectra of the same disease. PubMed. Ann
Dermatol Venereol. 2007 Jan;134(1):68-71. (Also see: Linear Scleroderma) |
| Morphea
of the breast. Two case reports Morphea is a recognised
sequelae of radiotherapy which should be distinguished from
sclerotic recurrence of the original tumour. Surgical excision
is possible in certain patients. PubMed. J Plast Reconstr
Aesthet Surg. 2006;59(10):1114-7. |
| 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) |
| Scleroderma:
a case report of possible cause of restricted movement of
the temporomandibular joint with effects on facial development. Localized
scleroderma is often benign, but may cause significant deformity,
if it occurs on the face or extends across joint surfaces.
Structural changes may occur in the osseous tissue and result
in mandibular joint restriction (pseudoankylosis) and facial
and occlusal disharmonies. PubMed. J Clin Pediatr Dent.
2003 Fall;28(1):33-8. (Also see: Linear,
and Dental
Involvement) |
| Associated Conditions |
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|
| Causes of Morphea |
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| Rare Types of Morphea |
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| Generalized Morphea |
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| Keloid Morphea |
|
| Morphea Profunda |
| Rare
Types of Morphea: Profunda. ISN. |
| Pansclerotic Morphea |
|
|
| Diagnosis of Morphea |
| Morphea is usually diagnosed by
clinical examination. It is often confirmed by skin biopsy.
Skin biopsies are usually a very tiny sample of tissue and
the procedure is often very quick, easy and with minimal discomfort.
Ultrasound has also been found to be useful for the diagnosis
of Localized Scleroderma (such as morphea). |
| ISN
Photo Repository: Morphea Photos. ISN. |
| A
new computerized method for the assessment of skin lesions in localized
scleroderma (LS). CSS (computerized skin score) has shown to be a reliable
method to assess the skin lesions in patients with LS. It is reproducible,
easy to use and, with the support of the CSS software, applicable worldwide. PubMed. Rheumatology (Oxford). 2007 Jan 30. (Also see: Localized
Scleroderma:Linear) |
| Rheumatoid
factor isotypes in localized scleroderma. The presence
of RF isotypes is one of the immunological abnormalities
of localized scleroderma. IgM RF seemed to be most useful
of these three factors to determine the severity of disease. PubMed.
Clin Exp Dermatol. 2005 Jul;30(4):405-8. |
| Juvenile-onset
localized scleroderma activity detection by infrared thermography. Our
results demonstrate that thermography is a promising diagnostic
tool when associated with clinical examination in discriminating
disease activity, as long as it is applied to lesions without
severe atrophy of the skin and subcutaneous fat. Rheumatology
(Oxford) 2002 Oct;41(10):1178-1182. PubMed. |
| Thirteen-megahertz
ultrasound probe: its role in diagnosing localized scleroderma. Thirteen-megahertz
ultrasound is a valuable tool for diagnosing LS. Morphological
ultrasound diagnostic criteria had a high specificity and
a high sensitivity. PubMed. Br J Dermatol 2003 Apr;148(4):724-9.
(Also see: Linear) |
| Antiphospholipid
antibody in localised scleroderma. These results suggest
that aCL (antibodies against cardiolipin) and LAC (lupus
anticoagulant) are the major autoantibodies in patients with
generalised morphoea. PubMed. Ann Rheum Dis. 2003 Aug;62(8):771-4.
(Also see: Antiphospholipid
Syndrome and Antibodies) |
| Anti-DNA
Topoisomerase II Alpha Autoantibodies in Localized Scleroderma. The
present results indicate that anti-topo II alpha Ab is a
major autoantibody in LSc, which is distinct from anti-topo
I Ab in SSc. Ikuko Hayakawa. ACR Conference Oct. 2003
(Also see: Localized
Scleroderma: Linear;
and Antibodies) |
| Serum
levels of manganese superoxide dismutase in patients with
localized scleroderma. The results suggested that the
serum levels of this enzyme may be a serological marker for
the disease activity and the extent of skin involvement in
this disease. PubMed. Exp Dermatol. 2004 Jun;13(6):357-60. |
| Treatment of Morphea |
|
|
| English Morphea Patient
and Caregiver Stories |
| Morphea
Patient and Caregiver Stories (Main Listing). ISN. |
| Italian Morphea Patient
and Caregiver Stories |
| Sclerodermia
dalla A alla Z: Morphea Storia. ISN. |
| Spanish Morphea Patient
and Caregiver Stories |
| La
Esclerodermia desde la A hasta la Z: Morfea Historia. ISN. |
| See Also |
| Juvenile
scleroderma. Scleroderma is a relatively rare disorder
in children. Among its subsets, localized scleroderma is
more common in children than the systemic variety. Curr
Opin Rheumatol 2002 Sep;14(5):553-61,PubMed. |
| Atrophoderma
of Pasini and Pierini (APP) is similar to Morphea Scleroderma.
Some doctors think it represents a late-stage Morphea. ISN. |
| Diseases
Similar to Scleroderma. ISN. |
| Juvenile
Scleroderma. ISN. |
| Lichen
Sclerosus. ISN. |
| Scleroderma
Photos. ISN. |
| Skin
Rashes and Other Changes. The location, appearance and
color of a rash will help your doctor make the diagnosis.
Look for care suggestions on this chart for common rashes. familydoctor.org. |
| References |
| [1] Morphea:
Clinical History. Occasionally, localized morpheic lesions
occur in the uninvolved skin of patients with systemic sclerosis.
Morphea has also been reported in association with systemic
lupus erythematosus, primary biliary cirrhosis, pemphigus,
and dermatomyositis. eMedicine.com. (Also see: Systemic
Scleroderma, Lupus, Primary
Biliary Cirrhosis, and Dermatomyositis.) |
| [2] Localized
scleroderma in adults and children. Clinical and laboratory
investigations on 239 cases. Children and adults developed
LS with analogous clinical and immunological features. However,
the prevalence of LS variants differed between adult and
pediatric populations, leading to different extracutaneous
complications. Among adults, Raynaud's phenomenon was found
in 8 patients; interestingly, anticentromere antibodies
were detected in 4 of these subjects, identifying a subset
at risk for progression to systemic
disease. PubMed. Eur J Dermatol 2003 Mar-Apr;13(2):171-6.
(Also see: Raynaud's and Linear
Scleroderma) |
| [3] Is
Juvenile Localized Scleroderma really "LOCALIZED"? One
fourth of JLS patients in this data series presented various
kind of extra-cutaneous manifestations, sometimes with multiorgan
involvement. For this reason, the term "localized" is
somehow inappropriate. These findings should change our clinical
approach to this disease and underline the need for systemic
immunosuppressive treatment for some patients. Francesco
Zulian. ACR Conference Oct. 2003. (Also see: Linear) |
| U.S. Morphea Registry and DNA Repository |
| First U.S. Morphea
Registry and DNA Repository for both Adults and Children Established. Dermatologists at University of Texas Southwestern Medical Center are establishing a DNA repository
aimed at people with morphea. They will collect information about other health
conditions present, collect information about family history, and collect
blood and skin samples to further define the genes associated with morphea
and the genetic faces of morphea. Southwestern Medical Center. 05/31/07. |
