単純性血管腫、ポートワイン様血管腫、ポートワイン母斑 portwine mark

WordNet

(microscopy) a dye or other coloring material that is used in microscopy to make structures visible
color for microscopic study; "The laboratory worker dyed the specimen"
color with a liquid dye or tint; "Stain this table a beautiful walnut color"; "people knew how to stain glass a beautiful blue in the middle ages"
produce or leave stains; "Red wine stains the table cloth"
a soiled or discolored appearance; "the wine left a dark stain" (同)discoloration, discolouration
marked or dyed or discolored with foreign matter; "a badly stained tablecloth"; "tear-stained cheeks"
having a coating of stain or varnish (同)varnished
the act of spotting or staining something (同)spotting, maculation
(histology) the use of a dye to color specimens for microscopic study

PrepTutorEJDIC

(…で)…‘を'『汚す』,‘に'しみをつける《+『名』+『with』+『名』》 / 〈ガラス・木材・布など〉‘に'『着色する』 / (…で)〈人格・名声など〉‘を'汚す,‘に'傷をつける《+『名』+『with』+『名』》 / 汚れる,しみがつく / 〈C〉〈U〉(…についた)『しみ』,汚れ《+『on』+『名』》 / 〈U〉〈C〉着色剤,染料 / 〈C〉(人格・名声などに対する)汚点,傷《+『on』(『upon』)+『名』》

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/02 22:16:55」(JST)

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A port-wine stain or nevus flammeus is almost always a birthmark.^[1] It is caused by a vascular anomaly (a capillary malformation in the skin).^[2]

Port-wine stains ordinarily persist throughout life.^[3] The area of skin affected grows in proportion to general growth. Port-wine stains occur most often on the face but can appear anywhere on the body, particularly on the neck and upper trunk.^[1]^[4] Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish color.^[1] In adulthood, thickening of the lesion or the development of small lumps may occur.^[1]^[5] They are called port wine for their reddish color.

Port-wine stains may be part of a syndrome such as Sturge–Weber syndrome or Klippel–Trénaunay–Weber syndrome.^[1]

Types

A hand with a red port-wine stain

Nevus flammeus may be divided as follows:^[6]

Nevus flammeus nuchae
Midline nevus flammeus

Genetics

Port-wine stains were shown to be caused by a somatic activating c.548G→A mutation in the GNAQ gene.^[7] An association with RASA1 has also been described.^[8]

Diagnosis

A healthcare provider can usually diagnose a port-wine stain based entirely upon the history and appearance. In unusual cases, a skin biopsy may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull.

A MRI scan of the brain may be performed (under anesthesia) on infants who have a port-wine stain in the head area in order to check for signs of Sturge-Weber syndrome.^[1]

If the port-wine stain is inside the mouth, a provider may check the insides of a newborn baby's throat with a scope to see if there are any changes (growths) other than just the color.

If the port-wine stain is around the eye or on the eyelid, a referral may be made to an optometrist or ophthalmologist for a test of the ocular pressures in that eye. If swelling occurs in the port-wine stain, it may cause vision problems, glaucoma, or blindness.

Treatment

Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing; port-wine stains can also be covered with cosmetics. Lasers may be able to destroy the capillaries without significant damage to the overlying skin. Lasers and other light sources may therefore be able to reduce the redness of stains, although there is not enough evidence to recommend one form over another. For most people in trials of pulsed dye laser, more than 25% of the redness was reduced by laser after one to three treatments. Adverse effects were rare in these trials, although some people had changes to the color of the skin, especially Chinese people with darker skin. There can be pain, crusting, and blistering in the two weeks after treatment. The trials only followed people for six months, so long-term outcomes are not known.^[2] Up to ten treatments may be necessary for improvement, but complete removal may not result.^[3]

The use of topical rapamycin as an adjunct to pulsed dye laser may improve results. ^[9]

Treatment is generally given before one year of age.^[10] However, as it is recommended to be performed under anesthesia (15 minutes) on small children, it is not always possible to get frequent treatments. For example in Finland a child gets treated 2-3 times per year, resulting in a target of "being ready before school age" (7 years).

After the laser treatment the skin is filled with black marks, the size of a pen. This is due to the laser instrument's size; the black marks disappear within 1–3 weeks. The treated area can be sore and swollen for a couple of days.

Prognosis

In the absence of successful treatment, hypertrophy (increased tissue mass) of the stains may cause problems later in life, such as loss of function (especially if the stain is near the eye or mouth), bleeding, and increasing disfigurement. Lesions on or near the eyelid can be associated with glaucoma.^[1] If the port-wine stain is on the face or other highly visible part of the body, its presence can also cause emotional and social problems for the affected person.

It is suggested that gene therapy might be used as a cure in the future.

Epidemiology

Studies have recorded an incidence of about 3–5 cases per 1,000 newborn babies.^[11]^[12]^[13]

References

^ ^a ^b ^c ^d ^e ^f ^g Primary Care Dermatology Society, UK. "Port-wine stain". Clinical Guidance. Primary Care Dermatology Society, UK. Retrieved 8 July 2013.
^ ^a ^b Faurschou, A.; Olesen, AB; Leonardi-Bee, J.; Haedersdal, M. (9 November 2011). "Lasers or light sources for treating port-wine stains.". Cochrane database of systematic reviews (Online) (11): CD007152. doi:10.1002/14651858.CD007152.pub2. PMID 22071834.
^ ^a ^b Patel, AM; Chou, EL; Findeiss, L; Kelly, KM (Jun 2012). "The horizon for treating cutaneous vascular lesions.". Seminars in cutaneous medicine and surgery 31 (2): 98–104. doi:10.1016/j.sder.2012.02.001. PMID 22640429.
^ Ryan, E; Warren, L (May 2012). "Birthmarks--identification and management.". Australian family physician 41 (5): 274–277. PMID 22558616.
^ Minkis, K.; Geronemus, R. G.; Hale, E. K. (2009). "Port wine stain progression: A potential consequence of delayed and inadequate treatment?". Lasers in surgery and medicine 41 (6): 423–6. doi:10.1002/lsm.20788. PMID 19588535.
^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 582–3. ISBN 0-7216-2921-0.
^ Shirley, Matthew D.; Tang, Hao; Gallione, Carol J.; Baugher, Joseph D.; Frelin, Laurence P.; Cohen, Bernard; North, Paula E.; Marchuk, Douglas A.; Comi, Anne M.; Pevsner, Jonathan (8 May 2013). "Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation in". New England Journal of Medicine 368 (21): 1971–9. doi:10.1056/NEJMoa1213507. PMID 23656586. |accessdate= requires |url= (help)
^ Eerola I, Boon LM, Mulliken JB et al. (December 2003). "Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations". Am. J. Hum. Genet. 73 (6): 1240–9. doi:10.1086/379793. PMC 1180390. PMID 14639529.
^ Marques L, Nunez-Cordoba JM, Aguado L, et al. Topical rapamycin combined with pulsed dye laser in the treatment of capillary vascular malformations in Sturge-Weber syndrome: phase II, randomized double-blind, intraindividual placebo controlled trial. JAAD 2015.
^ McLaughlin, MR; O'Connor, NR; Ham, P. (1 January 2008). "Newborn skin: Part II. Birthmarks.". American family physician 77 (1): 56–60. PMID 18236823.
^ Jacobs, A. H.; Walton, R. G. (1976). "The incidence of birthmarks in the neonate". Pediatrics 58 (2): 218–22. PMID 951136.
^ Alper, J. C.; Holmes, L. B. (1983). "The incidence and significance of birthmarks in a cohort of 4,641 newborns". Pediatric dermatology 1 (1): 58–68. doi:10.1111/j.1525-1470.1983.tb01093.x. PMID 6679890.
^ Dr Thajudheen MD (Dr Thaj laser skin hair clinic), Kannangath Jyothy, Arul Priyadarshini, "Treatment of port-wine stains with flash lamp pumped pulsed dye laser on Indian skin: A six year study", Dr. Thaj Laser Skin-Hair Clinic, 2 April 2014

External links

Information about Port Wine Stains from Children's Hospital, Seattle.
Birthmarks.com
Sturge–Weber Syndrome Community

UpToDate Contents

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1. 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
2. スタージウェーバー症候群 sturge weber syndrome
3. 新生児における血管病変 vascular lesions in the newborn
4. 皮膚血管病変に対するレーザーおよび光線療法 laser and light therapy for cutaneous vascular lesions
5. 幼児血管腫の疫学、病因、臨床的特徴、および合併症 epidemiology pathogenesis clinical features and complications of infantile hemangiomas

English Journal

Spatial frequency domain imaging of port wine stain biochemical composition in response to laser therapy: A pilot study.

Mazhar A, Sharif SA, Cuccia JD, Nelson JS, Kelly KM, Durkin AJ.SourceBeckman Laser Institute, University of California, Irvine, California 92612.
Lasers in surgery and medicine.Lasers Surg Med.2012 Aug 21. doi: 10.1002/lsm.22067. [Epub ahead of print]
BACKGROUND AND OBJECTIVE: Objective methods to assess port wine stain (PWS) response to laser treatment have been the subject of various research efforts for several years. Herein, we present a pilot study using a newly developed, light emitting diode (LED) based spatial frequency domain imaging (SF
PMID 22911574

Letter regarding "enhanced port-wine stain lightening achieved with combined treatment of selective photothermolysis and imiquimod".

Lister T.SourceWessex Specialist Laser Centre, Salisbury District Hospital, Salisbury, UK.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.].Dermatol Surg.2012 Aug;38(8):1414-5. doi: 10.1111/j.1524-4725.2012.02492.x.
PMID 22861064

Japanese Journal

正常adenosine deaminase(ADA)活性を伴う46, XX, del(20)(q11.23q13.11)の1例

園田徹,富森美絵子,岩城哲 [他訳],池脇信直,岩本壮太郎
九州保健福祉大学研究紀要 8, 117-121, 2007-03-25
46, XX, del(20)(q11.23q13.11)の1例を報告する。症例は在胎41週1日で出生した。出生体重は3, 082g、身長51.0cm、頭囲34.2cmであった。眼球陥凹、前向き鼻孔、前額部のポートワインマーク、耳介下方付着、両側軸三叉線高位、右股関節脱臼、全身の筋緊張低下などの異常がみられた。反復性感染の既往はなかった。生後7カ月のときの発達指数は約60であった。末梢血リンパ球を …
NAID 110006407501

可変式ロングパルスダイレーザー(Vビーム^【○!R】)の臨床的有用性

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日本レーザー医学会誌 = The Journal of Japan Society for Laser Medicine 27(4), 270-279, 2007-01-15
NAID 10018697438

先天性血管腫罹患者に見られた経験の意味づけ : 顔の変形を有する人への支援のニーズを探る

松本学
京都大学大学院教育学研究科紀要 50, 158-171, 2004-03-00
NAID 110000941273

Related Pictures

★リンクテーブル★

リンク元	「血管腫」「単純性血管腫」
関連記事	「stain」

「血管腫」

Nevus flammeus
	Mikhail Gorbachev has a prominent port-wine stain on his forehead.
Classification and external resources
Specialty	medical genetics
ICD-10	Q82.5 (ILDS Q82.510)
ICD-9-CM	757.32
OMIM	163000
DiseasesDB	10384
MedlinePlus	001475
eMedicine	derm/295
MeSH	D019339