WordNet

stop filming; "cut a movie scene"
cease, stop; "cut the noise"; "We had to cut short the conversation" (同)cut_off
a wound made by cutting; "he put a bandage over the cut" (同)gash, slash, slice
a share of the profits; "everyone got a cut of the earnings"
a step on some scale; "he is a cut above the rest"
grow through the gums; "The new tooth is cutting"
have grow through the gums; "The baby cut a tooth"
the act of cutting something into parts; "his cuts were skillful"; "his cutting of the cake made a terrible mess" (同)cutting
the act of penetrating or opening open with a sharp edge; "his cut in the lining revealed the hidden jewels" (同)cutting
the division of a deck of cards before dealing; "he insisted that we give him the last cut before every deal"; "the cutting of the cards soon became a ritual" (同)cutting
the act of shortening something by chopping off the ends; "the barber gave him a good cut" (同)cutting, cutting off
a trench resembling a furrow that was made by erosion or excavation (同)gash
(sports) a stroke that puts reverse spin on the ball; "cuts do not bother a good tennis player" (同)undercut
a canal made by erosion or excavation
an unexcused absence from class; "he was punished for taking too many cuts in his math class"
the act of reducing the amount or number; "the mayor proposed extensive cuts in the city budget"
a piece of meat that has been cut from an animal carcass (同)cut of meat
a distinct selection of music from a recording or a compact disc; "he played the first cut on the cd"; "the title track of the album" (同)track
(film) an immediate transition from one shot to the next; "the cut from the accident scene to the hospital seemed too abrupt"
the style in which a garment is cut; "a dress of traditional cut"
create by duplicating data; "cut a disk"; "burn a CD" (同)burn
(of a male animal) having the testicles removed; "a cut horse" (同)emasculated, gelded
weed out unwanted or unnecessary things; "We had to lose weight, so we cut the sugar from our diet" (同)prune, rationalize, rationalise
with parts removed; "the drastically cut film" (同)shortened
intentionally fail to attend; "cut class" (同)skip
(used of rates or prices) reduced usually sharply; "the slashed prices attracted buyers" (同)slashed
style and tailor in a certain fashion; "cut a dress" (同)tailor
mixed with water; "sold cut whiskey"; "a cup of thinned soup" (同)thinned, weakened
(of pages of a book) having the folds of the leaves trimmed or slit; "the cut pages of the book"
allow incision or separation; "This bread cuts easily"
discharge from a group; "The coach cut two players from the team"
dissolve by breaking down the fat of; "soap cuts grease"
divide a deck of cards at random into two parts to make selection difficult; "Wayne cut"; "She cut the deck for a long time"
fashioned or shaped by cutting; "a well-cut suit"; "cut diamonds"; "cut velvet"
fell by sawing; hew; "The Vietnamese cut a lot of timber while they occupied Cambodia"
form by probing, penetrating, or digging; "cut a hole"; "cut trenches"; "The sweat cut little rivulets into her face"
form or shape by cutting or incising; "cut paper dolls"
function as a cutting instrument; "This knife cuts well"
give the appearance or impression of; "cut a nice figure"
have a reducing effect; "This cuts into my earnings"
hit (a ball) with a spin so that it turns in the opposite direction; "cut a Ping-Pong ball"
make a recording of; "cut the songs"; "She cut all of her major titles again"
make an abrupt change of image or sound; "cut from one scene to another"
make an incision or separation; "cut along the dotted line"
move (ones fist); "his opponent cut upward toward his chin"
pass directly and often in haste; "We cut through the neighbors yard to get home sooner"
pass through or across; "The boat cut the water"
penetrate injuriously; "The glass from the shattered windshield cut into her forehead"
perform or carry out; "cut a caper"
reap or harvest; "cut grain"
record a performance on (a medium); "cut a record"
separate with or as if with an instrument; "Cut the rope"
separated into parts or laid open or penetrated with a sharp edge or instrument; "the cut surface was mottled"; "cut tobacco"; "blood from his cut forehead"; "bandages on her cut wrists"
shorten as if by severing the edges or ends of; "cut my hair"

PrepTutorEJDIC

(刃物などで)…'を'『切る』,傷つける / (刃物などで)…'を'『切断する』,切り分ける;〈草・作物・髪など〉'を'刈り取る / 〈像・文様など〉'を'『刻む』,彫る;〈石・宝石など〉'を'刻む;みがく;〈衣服など〉'を'裁断する / 《比喩(ひゆ)的に》〈人,人の心〉'を'切る,傷つける;〈寒さなどが〉…‘の'身を切る / (…から)…'を'『削除する』《+『名』+『from』(『out of』)+『名』》 / …'を'減らす,削減する;(…で)…'を'薄める《+『名』+『with』+『名』》 / …'を'横切る,‘と'交差する / …'を'掘る,切り開く / …'を'止める / 〈歯〉'を'はやす / 《話》(わざと)…'を'知らないふりをする,のけものにする / 《話》〈授業など〉'を'さぼる,欠席する / (カードゲームで)〈一組の札〉'を'切る / (テニス・卓球などで)〈ボール〉'を'カットする / 《しばしば状態を表す副詞[句]を伴って》〈刃物が〉『切れる』;〈物が〉切られる / 〈物事が〉身を切るように痛い;感情をひどく傷つける / (…へ)急に方向を変える《+『to』+『名』》 / (…への)切り傷,刻み目《+『on』(『in』)+『名』》 / (刃物の)一切り,(むちなどの)一打ち / (…の)断片(特に,切り取った肉など),切り身《+『of』+『名』》 / (…の)削減,(値段の)割引き,(賃金の)引下げ《+『in』+『名』》 / (…の)切り方,裁ち方,刈り方《+『of』+『名』》 / (テニス・卓球などで)ボールを切ること / 横断路,通り抜け路 / 切り通し;掘り割り / 木版[画];(印刷用の)金属板;さし絵,カット / (…に対する)感情を傷つける言葉(行為);しんらつな言葉,冷酷な仕打ち《+『at』+『名』》 / 《話》分け前,配前(share)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/10/30 00:04:21」(JST)

wiki en

Cutis marmorata telangiectatica congenita or CMTC is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. The condition was first recognised and described in 1922 by Cato van Lohuizen,^[1] a Dutch pediatrician whose name was later adopted in the other common name used to describe the condition - Van Lohuizen Syndrome. CMTC is also used synonymously with congenital generalized phlebectasia, nevus vascularis reticularis, congenital phlebectasia, livedo telangiectatica, congenital livedo reticularis and Van Lohuizen syndrome.^[2]

It should not be confused with the more general term "cutis marmorata", which refers to livedo reticularis caused by cold.^[3]

Eponym[edit]

It is named for Dr. Cato van Lohuizen.^[4]

Etiology and causes[edit]

Fewer than 100 cases of CMTC have been published worldwide. Petrozzi reported the first case of CMTC in the United States in 1970.^[5] CMTC is believed to be more common than suspected, as studies have shown that milder forms of the disease are not being recognized as CMTC.^[6]

The pathophysiology is still unclear, with most cases occurring sporadically, although rare cases were reported in families. Studies indicated the primary involvement of capillaries, venules and veins, and possibly also that of arterioles and lymphatics.

Hypotheses that have been proposed include: environmental/external factors;^[7] peripheral neural dysfunction;^[8] failure of the development of mesodermic vessels in an early embryonic stage; autosomal dominant inheritance with incomplete penetrance and, finally, the theory of Happle.^[8]

Symptoms and diagnosis[edit]

CMTC is an uncommon, sporadic congenital vascular malformation characterized by a generalized or localized reticulated cutaneous vascular network.

Usually observed at birth or shortly thereafter in 94% of patients,^[7] in other reports, patients did not develop skin lesions until 3 months or even 2 years after birth.^[9]^[10] Females are typically affected more often than males (64%).^[11]

Cutaneous lesions described in patients with CMTC include nevus flammeus, hemangioma, nevus anemicus, café-au-lait spots, melanocytic nevus, aplasia cutis and acral cyanosis.^[6]

It has a marbled bluish to deep-purple appearance. The dark skin lesions often show a palpable loss of dermal substance. The reticulated mottling frequently appears more prominent in a cold environment (physiologic cutis marmorata), but tends not to disappear with warming. Hence, the erythema may be worsened by cooling, physical activity, or crying.

CMTC frequently involves the extremities, with the lower extremities involved most commonly, followed by the upper extremities, and then the trunk and face. The lower extremities often show atrophy and seldom show hypertrophy resulting in limb circumference discrepancy.

When located on the trunk, the lesions of CMTC tend to show mosaic distribution in streaks with a sharp midline demarcation seen across the abdomen.^[6] The lesions are primarily localized, but can be segmental or generalized, often unilateral in appearance.^[6] Diffuse involvement of the skin is usually not observed.

Although its course is variable, the majority of lesions in mild cases fade by adolescence. Ulceration and secondary infection are complications in severe cases and can be fatal if present in the neonatal period.^[12] Recently, Melani et al.^[13] reported the case of a 20-year-old man with CMTC in association with chronic urticaria.

Differential diagnosis[edit]

During the first few weeks after birth, when the lesions are not very reticulated, CMTC may look very similar to vascular lesions such as port-wine stains. However, during follow-up, CMTC lesions become characteristic in their appearance. They must be differentiated from other causes of persistent reticulated vascular lesions, such as those in the following table:

Diseases	Characteristics
Diffuse phlebectasia	rare progressive harmartomatous malformation involving the deeper veins
Livedo reticularis associated with collagen-vascular disease	lace pattern of cyanotic skin discoloration secondary to dilation of subpapillary veinous plexi and occlusion of small vessels feeding the upper cutis
Neonatal lupus erythematosus	well-demarcated erythematous, mild-scaling plaque that is often annular and appears predominantly on the scalp, neck, or face
Nevus anemicus	congenital single patch manifested by skin pallor, most commonly seen on the trunk
Nevus flammeus (port-wine stain)	pale pink to red-purple, usually unilateral macules of the face or extremities
Physiologic cutis marmorata	reticulated mottling appearance of the skin that physiologically responds to cold environments
Primary antiphospholipid syndrome (APS)	increased tendency to form venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies

Histology[edit]

Some patients have a few or no histopathologic abnormalities. Histological examination of a biopsy may show an increase in the number and size of capillaries and veins (rarely lymphatics), dilated capillaries located in the deeper dermis, and hyperplasia and swollen endothelial cells with occasional dilated veins and venous lakes.

Associated abnormalities[edit]

Associated abnormalities include the following: • Body asymmetry (extremities; macrocephaly) • Glaucoma • Cutaneous atrophy • Neurological anomalies • Vascular anomalies (nevus flammeus /Sturge-Weber/Klippel-Trénauna Adams Oliver syndrome) • Psychomotor and/or mental retardation • Chronic ulceration that can complicate long-term CMTC • Chronic urticaria.^[13]

Treatment and complications[edit]

In general, there is no treatment available for CMTC, although associated abnormalities can be treated. In the case of limb asymmetry, when no functional problems are noted, treatment is not warranted, except for an elevation device for the shorter leg.

Laser therapy has not been successful in the treatment of CMTC, possibly due to the presence of many large and deep capillaries and dilated veins. Pulsed-dye laser and long-pulsed-dye laser have not yet been evaluated in CMTC, but neither argon laser therapy nor YAG laser therapy has been helpful.^[14]

When ulcers develop secondary to the congenital disease, antibiotic treatment such as oxacillin and gentamicin administered for 10 days has been prescribed. In one study, the wound grew Escherichia coli while blood cultures were negative.^[12]

Prognosis[edit]

The prognosis is favorable in most patients with an isolated cutaneous abnormality. In the majority of cases, both the vivid red marking and the difference in circumference of the extremities regress spontaneously during the first year of life. It is theorized that this may be due to the normal maturation process, with thickening of the epidermis and dermis. Improvements for some patients can continue for up to 10 years, while in other cases, the marbled skin may persist for the patient's lifetime.

One study reported an improvement in lesions in 46% of patients within 3 years.^[7] If CMTC persists into adulthood, it can result in complaints due to paresthesia, increased sensitivity to cold and pain, and the formation of ulcers.^[12]

Few reports included long-term follow up of CMTC into adolescence and adulthood. While about 50% of patients seem to show definite improvement in the reticular vascular pattern,^[14] the exact incidence and cause of persistent cases are unknown.

Psychological effects[edit]

Sufferers with visible marks generally feel fine (physically) and can act normally, but when it is mentioned, they may become withdrawn and self-conscious. Some children may have low self esteem due to the condition.

Clinical and patient support[edit]

Due to the very rare occurrence of the condition and its apparent neutrality to treatment (CMTC as a vascular, hereditary/gene disorder has no medicinal cure) clinical interest from the medical or pharmaceutcial fraternity has been limited when compared to conditions affecting larger numbers of the global population. As a result, and until recently, much of the clinical research carried out on the disease was either not widely published or accessible and CMTC sufferers, parents and newly diagnosed patients often resorted to independent support networks to seek clinical advice. In Holland, home to Cato van Lohuizen who first described Van Lohuizen Syndrome, Lex van der Heijden is now the President of www.cmtc.nl which is the globally recognised center for online patient and family support with a database of USA and European members.

References[edit]

^ Van Lohuizen CHJ. Cutis marmorata telangiectatica congenita,. Acta Derm Venereol. (Stockh). 1922:3:202-11.
^ Gerritsen MJ, et al. Cutis marmorata telangiectatica congenita: report of 18 cases. Br J Dermatol. 2000 Feb;142(2):366-9.
^ "cutis marmorata" at Dorland's Medical Dictionary
^ doctor/3295 at Who Named It?
^ Petrozzi JW, Rahn EK, Mofenson H, et al. Cutis marmorata telangiectatica congenita. Arch Dermatol. 1970 Jan;101(1):74-7.
^ ^a ^b ^c ^d Torrelo A, Zambrano A, Happle R. Cutis marmorata telangiectatica congenita and extensive mongolian spots: type 5 phacomatosis pigmentovascularis. Br J Dermatol. 2003 Feb;148(2):342-5.
^ ^a ^b ^c Amitai DB, Fichman S, Merlob P, et al. Cutis marmorata telangiectatica congenita: clinical findings in 85 patients. Ped Dermatol. 2000 Mar-Apr;17(2):100-4.
^ ^a ^b Bormann G, Wohlrab J, Fischer M, et al. Cutis marmorata telangiectatica congenita: laser doppler fluxmetry evidence for a functional nervous defect. Ped Dermatol. 2001Mar-Apr;18(2):110-3.
^ Powel ST, Su WP. Cutis marmorata telangiectatica congenita: report of nine cases and review of the literature. Cutis. 1984 Sep;34(3):305-12. Review.
^ Lee S, Lee JB, Kim JH, et al. Cutis marmorata telangiectatica congenita with multiple congenital anomalies (van Lohuizen’s syndrome). Dermatologica. 1981;163(5):408-12.
^ Vascular Lesions and Congenital Nevi in the Newborn. UpToDate viewed on 03-28-2008. http://www.utdol.com/utd/content/topic.do?topicKey=ped_derm/4439&type=P&selectedTitle=1~1.
^ ^a ^b ^c Hu IJ, Chen MT, Tai HC, et al. Cutis marmorata telangiectatica congenita with gangrenous ulceration and hypovolaemic shock. Eur J Pediatr. 2005 Jul;164(7):411-3.
^ ^a ^b Melani L, Antiga E, Torchia D, et al. Cutis marmorata telangiectatica congenita and chronic autoimmune urticaria in a young man. J Dermatol. 2007 Mar;34(3):210-3.
^ ^a ^b Mazereeuw-Hautier J, Carel-Caneppele S, Bonafe JL. Cutis marmorata telangiectatica congenita: report of two persistent cases. Ped Dermatol. 2002 Nov-Dec;19(6): 506-9.

External links[edit]

CMTC Information and Support Website
Skin and Aging
DermIS
PeaceHealth

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 新生児における血管病変 vascular lesions in the newborn
2. 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
3. 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
4. 胎児性巨大児 fetal macrosomia
5. シャルコーマリートゥース病などの一次性の遺伝性運動感覚性ニューロパチー hereditary primary motor sensory neuropathies including charcot marie tooth disease

English Journal

Diagnosis and management of extensive vascular malformations of the lower limb: part II. Systemic repercussions [corrected], diagnosis, and treatment.

Redondo P, Aguado L, Martínez-Cuesta A.SourceUnit of Vascular Malformations, Department of Dermatology, University Clinic of Navarra, Pamplona, Spain. predondo@unav.es
Journal of the American Academy of Dermatology.J Am Acad Dermatol.2011 Nov;65(5):909-23; quiz 924.
At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Park
PMID 22000871

Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.

Redondo P, Aguado L, Martínez-Cuesta A.SourceUnit of Vascular Malformations, Department of Dermatology, University Clinic of Navarra, Pamplona, Spain. predondo@unav.es
Journal of the American Academy of Dermatology.J Am Acad Dermatol.2011 Nov;65(5):893-906; quiz 907-8.
There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermato
PMID 22000870

Japanese Journal

症例血管腫・心奇形・先天性片眼白内障を伴った血管拡張性大理石様皮斑の1例

平井伸幸,田村政昭
皮膚科の臨床 52(6), 859-862, 2010-06
NAID 40017168250

Apnea and macrocephaly-cutis marmorata telangiectatica congenita

FRANKLIN Brodus,GASCO Jaime,RANGEL-CASTILLA Leonardo,NAUTA Haring J. W.
Brain & development 31(9), 706-709, 2009-10-01
NAID 10026412836

Related Pictures

★リンクテーブル★

リンク元	「神経皮膚症候群」
関連記事	「cut」「congenita」「telangiectatic」「cutis」

「神経皮膚症候群」

Cutis marmorata telangiectatica congenita
	Classification and external resources
ICD-10	L95.0
OMIM	219250
DiseasesDB	33782
MedlinePlus	001478
eMedicine	derm/793

　　[★]

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)