generalized morphea

WordNet

1. draw from specific cases for more general cases (同)generalise, extrapolate, infer
2. become systemic and spread throughout the body; "this kind of infection generalizes throughout the immune system" (同)generalise
3. speak or write in generalities (同)generalise
4. not biologically differentiated or adapted to a specific function or environment; "the hedgehog is a primitive and generalized mammal" (同)generalised
5. change shape as via computer animation; "In the video, Michael Jackson morphed into a panther"
6. cause to change shape in a computer animation; "The computer programmer morphed the image"
7. localized scleroderma

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2018/10/01 13:52:59」(JST)

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See also: Morphia (disambiguation) and Morphine

Morphea
Specialty	Dermatology

Morphea, also called localized scleroderma or circumscribed scleroderma, is a form of scleroderma that involves isolated patches of hardened skin on the face, hands, and feet, or anywhere else on the body, with no internal organ involvement.

Classification

• Morphea–lichen sclerosus et atrophicus overlap is characterized by both lesions of morphea and lichen sclerosus et atrophicus, most commonly seen in women.
• Generalized morphea is characterized by widespread indurated plaques and pigmentary changes, sometimes associated with muscle atrophy, but without visceral involvement.
• Morphea profunda involves deep subcutaneous tissue, including fascia, and there is a clinical overlap with eosinophilic fasciitis, eosinophilia-myalgia syndrome, and the Spanish toxic oil syndrome. Morphea profunda shows little response to corticosteroids and tends to run a more chronic debilitating course.
• Pansclerotic morphea is manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, the bone, all causing disabling limitation of motion of joints.
• Linear scleroderma is a type of localised scleroderma which is an autoimmune disease characterized by a line of thickened skin which can affect the bones and muscles underneath it. It most often occurs in the arms, legs, or forehead, and may occur in more than one area. It is also most likely to be on just one side of the body. Linear scleroderma generally first appears in young children.
• Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. Multiple lesions of en coup de sabre may coexist in a single patient, with one report suggesting that the lesions followed Blaschko's lines. It gets its name from the perceived similarity to a sabre wound.

Frontal linear scleroderma

• Atrophoderma of Pasini and Pierini (also known as "Dyschromic and atrophic variation of scleroderma," "Morphea plana atrophica," "Sclérodermie atrophique d'emblée") is a disease characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may be thickened. Atrophoderma of Pasini and Pierini affects less than 200,000 Americans and is classified as a rare disease by http://rarediseases.info.nih.gov...") The disease results in round or oval patches of hyper-pigmented skin. The darkened skin patches may sometimes have a bluish or purplish hue when they first appear and are often smooth to the touch and hairless.

Signs and symptoms

Frontal linear scleroderma

Morphea most often presents as macules or plaques a few centimeters in diameter, but also may occur as bands or in guttate lesions or nodules.

Morphea is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. Morphea discriminates from systemic sclerosis by its supposed lack of internal organ involvement. This classification scheme does not include the mixed form of morphea in which different morphologies of skin lesions are present in the same individual. Up to 15% of morphea patients may fall into this previously unrecognized category.

Cause

Physicians and scientists do not know what causes morphea. Case reports and observational studies suggest there is a higher frequency of family history of autoimmune diseases in patients with morphea. Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies. Case reports of morphea co-existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea as an autoimmune disease.

B burgdorferi infection may be relevant for the induction of a distinct autoimmune type of scleroderma; it may be called "Borrelia-associated early onset morphea" and is characterized by the combination of disease onset at younger age, infection with B burgdorferi, and evident autoimmune phenomena as reflected by high-titer antinuclear antibodies.

Treatment

Throughout the years, many different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids. Antimalarials such as hydroxychloroquine or chloroquine have been used. Other immunomodulators such as methotrexate, topical tacrolimus, and penicillamine have been tried. Some have tried prescription vitamin-D with success. Ultraviolet A (UVA) light, with or without psoralens have also been tried. UVA-1, a more specific wavelength of UVA light, is able to penetrate the deeper portions of the skin and thus, thought to soften the plaques in morphea by acting in two fashions:[1]

• 1) by causing a systemic immunosuppression from UV light.
• 2) by inducing enzymes that naturally degrade the collagen matrix in the skin as part of natural sun-aging of the skin.

As with all of these treatments for morphea, the difficulty in assessing outcomes in an objective way has limited the interpretation of most studies involving these treatment modalities.

Epidemiology

Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1. Morphea occurs in childhood as well as in adult life. Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. Adequate studies on the incidence and prevalence have not been performed. Morphea also may be under-reported, as physicians may be unaware of this disorder, and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist.

See also

• List of cutaneous conditions
• Frontal linear scleroderma (morphea en coup de sabre)

Further reading

• JAMA Dermatology Patient Page. Morphea (Localized Scleroderma. Nicole M. Fett, MD. JAMA Dermatol. 2013;149(9):1124. doi:10.1001/jamadermatol.2013.5079. September 2013

External links

Classification	D ICD-10: L94.0 ICD-9-CM: 701.0 MeSH: D012594 DiseasesDB: 8351
External resources	eMedicine: derm/272 Patient UK: Morphea

UpToDate Contents

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• 1. 成人における限局性強皮症の病因、臨床症状、および診断 pathogenesis clinical manifestations and diagnosis of morphea localized scleroderma in adults
• 2. 小児期の限局性強皮症 localized scleroderma in childhood
• 3. 強皮症の概要および分類 overview and classification of scleroderma disorders
• 4. 成人における斑状強皮症（限局性強皮症）の治療 treatment of morphea localized scleroderma in adults
• 5. 全身性硬化症（強皮症）の成人の前治療評価 pretreatment evaluation of adults with systemic sclerosis scleroderma

English Journal

• Generalized morphea with preceding severe pain and coexistent early primary biliary cirrhosis.

• Iga N1, Otsuka A1, Iwata M1, Ueda Y2, Kabashima K1.
• European journal of dermatology : EJD.Eur J Dermatol.2015 Jun 17. [Epub ahead of print]
• PMID 26055733

• Imatinib treatment of therapy resistant generalized deep morphea.

• Alcántara-Reifs CM1, Garnacho-Saucedo GM1, Salido-Vallejo R1, de la Corte-Sánchez S1, García-Nieto AV1.
• Dermatologic therapy.Dermatol Ther.2015 Jun 2. doi: 10.1111/dth.12248. [Epub ahead of print]
• PMID 26032018

Japanese Journal

• 左右対称性に広範な分布を呈し筋原性酵素の上昇を伴ったGeneralized Morpheaの1例 (特集 膠原病)

• 皮膚科の臨床 57(1), 23-27, 2015-01
• NAID 40020322468

• 症例報告 硬化性萎縮性苔癬様の病理組織像を示したgeneralized morpheaの1例

• 臨床皮膚科 = Japanese journal of clinical dermatology 68(11), 879-883, 2014-10
• NAID 40020218237

• 症例報告 汎発型限局性強皮症の1例

• 臨床皮膚科 67(12), 965-970, 2013-11
• NAID 40019862560

Related Links

• Generalized Morphea Scleroderma - Scleroderma? We can help!

Generalized morphea, symptoms, diagnosis, treatments, support and personal stories about this rare form of Localized Scleroderma. Generalized morphea usually begins as patches of yellowish or ivory-colored rigid, dry skin, but it ...

• GENERALIZED MORPHEA: A CASE REPORT - Journal of IMAB

patients with plaque-type morphea, in 75% of those with generalized morphea, and in 50% of those with linear morphea and these antibodies correlate with the extension and the activity of the disease, too. Anticentromere, anti ...

• Morphea (Localized Scleroderma) - Dermatology Advisor

Are You Confident of the Diagnosis? Demographics Morphea may present at any age. In children, the linear subtype predominates while in adults’ plaque and generalized subtypes are most common. Morphea occurs more frequently ...

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★リンクテーブル★

リンク元	「多発性モルヘア」
関連記事	「generalize」「generalized」

「多発性モルヘア」

　　[★]

英

generalized morphea

同

多発性斑状強皮症

「generalize」

　　[★]

• v.

• 一般化する、普遍化する、全身性化する

関

generalisation、generalise、generalization

　 　 　

「generalized」

　　[★]

• adj.

• 汎化の、全般的な、全般性の、全身性の

関

generalization、systemic、systemically