phacomatosis pigmentovascularis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/11/22 12:39:12」(JST)

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Phakomatosis pigmentovascularis refers to the coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.

See also

• List of cutaneous conditions

References

UpToDate Contents

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• 1. 新生児における血管病変 vascular lesions in the newborn
• 2. 毛細血管奇形（ポートワイン母斑）：臨床的特徴、診断、および関連する症候群 capillary malformations port wine stains clinical features diagnosis and associated syndromes
• 3. もやもや病：病因、臨床的特徴、および診断 moyamoya disease etiology clinical features and diagnosis
• 4. 新生児および乳児における皮膚および頭皮の良性病変 benign skin and scalp lesions in the newborn and young infant

English Journal

• Phacomatosis melanorosea without extracutaneous features: an unusual type of phacomatosis pigmentovascularis.

• Arnold AW, Kleine MU, Happle R.SourceFreiburg University Medical Center, Department of Dermatology, Hauptstrasse 7, 79104 Freiburg, Germany. aarnold@uhbs.ch
• European journal of dermatology : EJD.Eur J Dermatol.2012 Jul-Aug;22(4):473-5. doi: 10.1684/ejd.2012.1780.
• Phacomatosis pigmentovascularis (PPV) represents a group of different types of didymosis (twin spotting) characterized by the coexistence of a large pigmentary nevus such as a mongolian spot, café-au-lait macule or macular nevus spilus, and an extensive telangiectatic nevus, such as nevus flammeus
• PMID 22713702

• Whorled hairless nevus of the scalp, linear hyperpigmentation, and telangiectatic nevi of the lower limbs: a novel variant of the "phacomatosis complex".

• Castori M, Scarciolla O, Morlino S, Manente L, Biscaglia A, Fragasso A, Grammatico P.SourceMedical Genetics, Department of Molecular Medicine, Sapienza University, San Camillo-Forlanini Hospital, Rome, Italy. mcastori@scamilloforlanini.rm.it
• American journal of medical genetics. Part A.Am J Med Genet A.2012 Feb;158A(2):445-9. doi: 10.1002/ajmg.a.34403. Epub 2012 Jan 13.
• The term "phacomatosis" refers to a growing number of sporadic genetic skin disorders characterized by the combination of two or more different nevi and possibly resulting from non-allelic twin spotting. While phacomatosis pigmentovascularis (PPV) and pigmentokeratotica represent the most common pat
• PMID 22247078

Japanese Journal

• Cutis marmorata telangiectatica congenita and extensive Mongolian spots : Type 5 phacomatosis pigmentovascularis

• TORRELO A
• Br J Dermatol 148, 342-345, 2003
• NAID 30014637839

• 色素血管母斑症に対する多種レーザー同時併用療法の経験

• 堀 圭二朗,河野 太郎,菊池 雄二,大久保 麗,岩坂 督,野崎 幹弘
• 日本レーザー医学会誌 = The Journal of Japan Society for Laser Medicine 23(2), 53-57, 2002-06-28
• NAID 10011171604

Related Links

• Phacomatosis pigmentovascularis | Disease | Office of Rare ...

If phacomatosis pigmentovascularis (PPV) is not associated with systemic complications (e.g., Sturge-Weber syndrome, Klippel-Trenaunay syndrome, eye conditions) it requires no treatment, however pulsed dye laser ...

• Phacomatosis pigmentovascularis | Disease | Overview | Office ...

Phacomatosis pigmentovascularis (PPV) is a skin and blood vessel disorder that is present from birth. Common signs and symptoms include port wine stain and pigmentary lesions, such as melanocytic nevi or epidermal ...

Related Pictures

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★リンクテーブル★

リンク元

「神経皮膚症候群」

「神経皮膚症候群」

　　[★]

英

neurocutaneous syndrome、neurocutaneous disorder

関

母斑症 phakomatosis

• 外胚葉異常により皮膚母斑や神経腫瘍を生じる先天性疾患

• 神経線維腫症1型, neurofibromatosis type 1, NF1, フォン・レックリングハウゼン病 von Recklinghausen disease：NF1の変異による常染色体優性遺伝疾患で多発性のカフェオレ斑、神経線維腫が特徴的。皮膚症状(カフェオレ斑、神経線維腫、黄色腫、貧血母斑、母斑性黄色内皮腫)、眼症状(リッシュ結節)

• 神経線維腫症2型, neurofibromatosis type 2, NF2
• 結節性硬化症, tuberous sclerosis
• ポイツ・イェーガー症候群, Peutz-Jeghers syndrome：顔面片側性の単純血管腫(三叉神経第1枝に沿う(CN V1))、眼の脈絡膜血管腫、脳軟膜の血管腫　→　中枢神経症状(乳幼児期からの痙攣発作、大脳半球萎縮、石灰化、知能障害)、眼症状(脈絡膜の血管腫による眼圧亢進、緑内障　→　牛眼　)
• 色素失調症, incontinentia pigmenti, ブロッホ・ザルツバーガー症候群, Bloch-Sulzberger syndrome
• スタージ・ウェーバー症候群, Sturge-Weber syndrome：遺伝性無し。皮膚症状：顔面片側性の単純血管腫(三叉神経第1枝に沿う(CN V1)) 。中枢神経症状：顔面血管腫が存在する側の特に後頭葉に脳軟膜の血管腫が発生 (NDE.349)。乳幼児期からの血管発作。大脳半球萎縮、石灰化、知能障害。眼症状：緑内障(牛眼)
• クリッペル・トレノーニイ・ウェーバー症候群, Klippel-Trenaunay-Weber syndrome
• 神経皮膚黒色症, neurocutaneous smelanosis
• 汎発性黒子症候群, lentiginosis profusa, LEOPARD症候群
• 基底細胞母斑症候群, basal cell nevus syndrome
• フォン・ヒッペル・リンドウ病, von Hippel-Lindau disease：常染色体優性遺伝。
• 色素血管母斑症, phacomatosis pigmentovascularis, オスラー病, Osler disease
• 遺伝性出血性毛細血管拡張症, hereditary hemorrhagic telangiectasia
• 青色ゴムマリ様母斑症候群, blue rubber-bleb nevus syndrome
• マフッチ症候群, Maffucci's syndrome
• Zinsser-Cole-Engman症候群, Zinsser-Cole-Engman syndrome
• 先天性角化異常症, dyskeratosis congenita
• 表皮母斑症候群, epidermal nervus syndrome
• 先天性血管拡張性大理石様皮斑, cutis marmorata telangiectatica congenita