Cutis marmorata telangiectatica congenita cmtc is a birth defect involving. Cutis marmorata telangiectatica congenita cmtc was reported first in 1922 by van lohuizen. Cutis marmorata telangiectatica congenita and aberrant. Cutis marmorata telangiectatica congenita cmtc is an uncommon, sporadic, congenital cutaneous condition presenting with persistent cutis marmorata, telangiectasia, phlebectasia and possible ulceration of the involved skin, skin atrophy, and undergrowth of the involved extremity. It should not be confused with cutis marmorata, a normal physiologic skin mottling in cool environments. Cutis marmorata telangiectatica congenita cmtc was first described by the dutch pediatrician van lohuizen 1 in 1922.
Cutis marmorata telangiectatica congenita cmtc is an uncommon congenital vascular malformation characterized by the presence of a localized or generalized pattern, frequently asymmetrical, with reticulated, erythematous or violaceous macules, present at birth or shortly thereafter. Cutis marmorata telangiectatica congeita cmtc boston. This case highlights the possibility of occurrence of multiple vascular malformations in the same patient. While physiological cutis marmorata is a frequently encountered condition, we report a rare entity that appears similar, but should not be confused with it. Cutis marmorata telangiectatica congenita cmtc is a rare condition that mainly affects the blood vessels of the skin. Cutis marmorata telangiectatica congenita 1 is a rare benign sporadic congenital vascular anomaly characterised by persistent cutis marmorata, telangiectasia, and phlebectasia and often associated with skin atrophy and ulceration. Here, we have compiled information on published cmtc patients with the aim to. Cutis marmorata telangiectatica congenita is a rare congenital cutaneous vascular anomaly consisting of a sharply demarcated, reticulated vascular network of blueviolet venules associated with telangiectasias, with or without varicose veins.
Cutis marmorata telangiectatica congenita cmtc is a rare condition that causes a marbled pattern on the skin. Cutaneous marbling is apparent at room temperature and becomes more pronounced with lower temperatures and crying. Review open access cutis marmorata telangiectatica congenita. Cutis marmorata telangiectatica congenita cmtc is a birth defect involving the skin and blood vessels. Cmtc is not caused by any known drug, medication or environmental factor that the baby may have been exposed to during the pregnancy. Cmtcovm cutis marmorata telangiectatica nord national. These dilated vessels cause the skin to have a marbled or fishnet appearance cutis mamorata. Cutis marmorata telangiectatica congenita cmtc is an uncommon, sporadic, congenital cutaneous vascular anomaly evident as persistent cutis marmorata, telangiectasia, and phlebectasia. Cutis marmorata telangiectatica congenita wikipedia. Type v phacomatosis pigmentovascularis or phacomatosis cesiomarmorata guilian du, ba,a xiaomin zhang, ms,b and tangde zhang, msb shenzhen and guangdong, china. It is a selflimiting disorder without associated anomalies, and its recovery is expected within the first 2 years of life.
Pdf cutis marmorata telangiectatica congenita and glaucoma. In some affected individuals, ulcerations or congenital. It is mostly cosmetic, and while it is present at birth, it fades considerably over a childs first year. Cutis marmorata telangiectatica congenita cmtc is a rare capillary malformation characterised by persistent reticulated marbled erythema. A 1dayold girl with lesions of cutis marmorata telangiectatica congenita that resemble nevi flammei. Cmtc is a disorder that is present at birth and is characterized by discolored patches of skin caused by dilated blood vessels livedoreticulartelangiectases. To further the current understanding of this disorder, we analyzed the clinical features of a large. Cutis marmorata telangiectatica congenita is a relatively uncommon condition, with about 300 cases described in the literature to date. Diagnosis is clinical, and there is no gold standard for. Cutis marmorata telangiectatica congenita cmtc is a rare capillary malformation characterised by. What is cutis marmorata telangiectatica congenita cmtc.
Cutis marmorata telangiectatica congenita cmtc is a rare, deep purple, marble or netlike birthmark. All but two patients were examined in the first year of life. This information sheet from great ormond street hospital gosh explains the causes, symptoms and treatment of cutis marmorata telangiectatica congenita cmtc and where to get help. In most cases, cmtc does not cause any problems and the skin marbled appearance fades in early childhood. When a newborn infant is exposed to low environmental temperatures, an evanescent, lacy, reticulated red andor blue cutaneous vascular pattern appears over most of the body surface. Cutis marmorata telangiectatica congenita cmtc is a rare congenital present at birthdisorder characterized by discolored patches of skin caused by widened dilated surface blood vessels. Concurrent sturgeweber syndrome, facial infantile hemangioma. Cutis marmorata telangiectatica congenita cmtc is a condition that is present at birth and causes discoloration of the skin due to dilated blood vessels visible near the surface.
Cutis marmorata telangiectasica congenita en rn del sexo femenino. Case report cutis marmorata telangiectatica congenita and aberrant mongolian spots. Cutis marmorata telangiectatica congenita clinical. Picture of cutis marmorata telangiectatica congenita. Cutis marmorata telangiectatica congenita in a female newborn. Cutis marmorata telangiectatica congenita is characterized by reticular erythema that is either generalized over the entire body or localized to a specific area or. Localized cutis marmorata telangiectatica congenita. Patients with cutis marmorata telangiectatica congenita should be. Mim219250 capillaryvenous cutaneous malformation with marbled appearance. Cutis marmorata telangiectatica congenita cmtc is a rare, sporadic, congenital cutaneous vascular disorder of unknown etiology. Cutis marmorata telangiectatica congenita cmtc is a rare vascular anomaly. To evaluate the distribution of skin lesions, clinical features, and associated abnormalities in children with cutis marmorata telangiectatica congenita at onset and during followup. Associated abnormalities such as body asymmetry, vascular.
The misnomer macrocephalycutis marmorata telangiectatica. Ocular manifestations of cutis marmorata telangiectatica congenita. Cutis marmorata telangiectatica congenita genetic and. Pdf cutis marmarata telangiectatica is a benign vascular anomaly that manifests as dilatation of superficial capilllaries and veins usually present at. It is characterized by patches of marbledlooking skin cutis marmarota, small widened blood vessels under the skin telangiectasia and varicose veins phlebectasia. Pdf cutis marmorata telangiectatica congenita cmtc is an uncommon, congenital, vascular malformation, characterised by a fixed, generalised or.
Cutis marmorata telangiectatica congenita cmtc is a rare, sporadic condition that presents at birth as a localised or generalised reticulated, blueviolet vascular network in the skin. A newborn female baby whose mother was aged 29 years and used propylthiouracil during pregnancy was hospitalized because of varicose lesions on the skin of the lower extremity and on the back, which were present at birth. Cmtc includes a mottled pattern on the surface of the skin. Cutis marmorata telangiectatica congenita nord national. Cutis marmorata telangiectatica congenita statpearls ncbi. They asserted that this association is best explained as a phenomenon of nonallelic twin spotting and suggested that this condition may be a novel variant form of phacomatosis pigmentovascularis.
Esterly, md chicago, illinois twentytwo cases of cutis marmorata telangiectatica congenita were evaluated during an 8year period. Localized cutis marmorata telangiectatica congenita suarez. Cutis marmorata telangiectatica congenita and glaucoma. Mar 25, 2019 cutis marmorata telangiectatica congenita cmtc is a birth defect involving the skin and blood vessels. Cutis marmorata telangiectatica congenita cmtc is a benign rare vascular anomaly often noted in the newborn period. Cutis marmorata telangiectatica congenita cmtc is an uncommon, sporadic, congenital cutaneous condition presenting with. It tends to be associated with cutaneous atrophy, ulcerations and body asymmetry. Cutis marmorata occurs in about 50% of children and is typically seen throughout infancy. Severe cmtc can create ulcerations in the affected skin area. Learn the symptoms, other problems it can cause, and how its treated. Jul 11, 2019 cutis marmorata telangiectatica congenita is a rare, benign, sporadic and cutaneous vascular disease. Who gets c utis marmorata telangiectatica congenita. A child with lesions of cutis marmorata telangiectatica congenita that are almost generalized. Cutis marmorata telangiectatica congenita is a lowflow vascular malformation that is characterized by its distinctive violaceous coarsely reticulated marbled marmorata pattern figure 3c.
Unlike cutis marmorata, it does not disappear with warming. Picture of skin diseases and problems cutis marmorata. We report a case of cmtc with atrophy, restricted in size to a small area on the back. Congenital glaucoma associated with cutis marmorata telangiectatica congenita. Cutis marmorata telangiectatica congenita cmtc is an uncommon congenital vascular malformation characterized by the presence of a localized or generalized. There have been about 300 cases reported to date in the. Cutis marmorata telangiectatica congenita with gangrenous ulceration and hypovolaemic. Hu ij, chen mt, tai hc, tsao pn, chou hc, hsieh ws. Cutis marmorata is distinct from cutis marmorata telangiectatica congenita cmtc. Pdf cutis marmorata telangiectatica congenita researchgate.
Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. It is defined as a localized or generalized reticulated, vascular, blueviolet network that is usually present at birth. The condition was first recognised and described in 1922 by cato van lohuizen, a dutch pediatrician whose name was later adopted in the other common name used to describe the condition van lohuizen syndrome. Cutis marmorata telangiectatica congenita is a relatively uncommon disorder that can mimic cutis marmorata, a common benign physiologic response to cold in infants. We present a unique case of 3 vascular malformationssturgeweber syndrome sws, facial infantile hemangioma ih, and cutis marmorata telangiectatica congenita cmtcwith dermatologic manifestations presenting in the same patient. Cutis marmorata telangiectatica congenita is most commonly localized. Here, we have compiled information on published cmtc patients. Description the cmtcovm is an organization based in the netherlands that provides information and other services to those affected with, or working on, van lohuizen syndrome, which is also known as cutis marmorata telangiectatica congenita cmtcovm. Cutis marmorata telangiectatica congenita is an uncommon congenital capillary vascular malformation. As a result, the skin has a purple or blue marbled or fishnet appearance cutis marmorata. Cutis marmorata telangiectatica congenita cmtc great.
Cutis marmorata telangiectatica congenita with gangrenous ulceration and hypovolaemic shock. Cutis marmorata telangiectatica congenita is a rare, benign, sporadic and cutaneous vascular disease. Cutis marmorata telangiectatica congenita an overview. Cutis marmorata telangiectatica congenita cmtc is a rare, usuaiiy sporadic, congenital, reticulated vascular anomaly, it may be widespread or segmental, with atrophy or ulceration, and may be associated with other congenital abnormalities. Cutis marmorata telangiectatica congenita cmtc is an uncommon congenital vascular malformation characterized by the presence of a localized or generalized pattern, frequently asymmetrical, with reticulated, erythematous or. Cutis marmorata telangiectatica congenita is clinically similar, but the lesions are more intense, may be segmental, are persistent, and may be associated with loss of dermal tissue, epidermal atrophy and ulceration.
Cutis marmorata telangiectatica congenita dermnet nz. Cutis marmorata telangiectatica congenita cmtc is a rare complex vascular malformation associated with a reticulated vascular pattern associated with dermal atrophy. The prognosis is usually good, with minor associated anomalies. Cutis marmorata telangiectatica congenita adc fetal. The discoloration may appear in a fishnet or marbled pattern. To further the current understanding of this disorder. This purplish pattern is similar to the fishnetlooking. Type v phacomatosis pigmentovascularis or phacomatosis cesiomarmorata guilian du, ba,a xiaomin zhang, ms,b and tangde zhang, msb.
Cutis marmorata telangiectatica congenita cmtc is a rare, sporadic condition that presents at birth as a localised or. This marbled pattern is always visible, but may be enhanced by cold temperatures or distress. The skin findings most often occur on the legs, but may also occur on the arms and trunk. Cutis marmorata telangiectatica congenita cmtc is a birthmark that affects the blood vessels in the skin causing a netlike pattern on the skin giving a marbled appearance. Cutis marmorata telangiectatica congenita definition of. Pdf to evaluate the distribution of skin lesions, clinical features, and associated abnormalities in children with cutis marmorata telangiectatica. Cmtc is a rare condition that mainly affects the blood vessels of the skin. Cutis marmorata telangiectatica congenita with skin. Cutis marmorata telangiectatica congenita genetic and rare.
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