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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. The lesions are primarily localized, but can be segmental or generalized, often unilateral in appearance. Diffuse involvement of the skin is usually not
274:
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
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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.
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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
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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
107:
People with visible marks generally feel fine (physically) and can act normally, but when it is mentioned, they may become withdrawn and self-conscious.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. CMTC is an uncommon, sporadic congenital vascular malformation characterized by a
77:, 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
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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
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Hypotheses that have been proposed include: environmental/external factors; peripheral neural dysfunction; failure of the development of mesodermic vessels in an early embryonic stage; autosomal dominant inheritance with incomplete penetrance and, finally, the theory of Happle.
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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
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Fewer than 100 cases of CMTC have been published worldwide. Petrozzi reported the first case of CMTC in the United States in 1970. 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.
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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.
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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, the exact incidence and cause of persistent cases are unknown.
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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.
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Usually observed at birth or shortly thereafter in 94% of patients, in other reports, patients did not develop skin lesions until three months or even two years after birth. Females are typically affected more often than males (64%).
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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.
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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
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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.
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For a full and up-to-date description visit the CMTC webpages of the global non-profit patient organisation for people with CMTC and other vascular malformation CMTC-OVM: www.cmtc.nl/en
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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.
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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.
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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.
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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
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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.
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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.
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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.
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One study reported an improvement in lesions in 46% of patients within three years. If CMTC persists into adulthood, it can result in complaints due to
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increased tendency to form venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies
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lace pattern of cyanotic skin discoloration secondary to dilation of subpapillary veinous plexi and occlusion of small vessels feeding the upper cutis
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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.
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well-demarcated erythematous, mild-scaling plaque that is often annular and appears predominantly on the scalp, neck, or face
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Gerritsen MJ, et al. Cutis marmorata telangiectatica congenita: report of 18 cases. Br J Dermatol. 2000 Feb;142(2):366-9.
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Petrozzi JW, Rahn EK, Mofenson H, et al. Cutis marmorata telangiectatica congenita. Arch
Dermatol. 1970 Jan;101(1):74-7.
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Van
Lohuizen CHJ. Cutis marmorata telangiectatica congenita,. Acta Derm Venereol. (Stockh). 1922:3:202-11.
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reticulated mottling appearance of the skin that physiologically responds to cold environments
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Vascular anomalies (nevus flammeus /Sturge-Weber/Klippel-Trénauna Adams Oliver syndrome)
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When ulcers develop secondary to the congenital disease, antibiotic treatment such as
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Cutis marmorata telangiectatica congenital is inherited via autosomal recessive manner
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congenital single patch manifested by skin pallor, most commonly seen on the trunk
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Vascular
Lesions and Congenital Nevi in the Newborn. UpToDate viewed on 03-28-2008
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pale pink to red-purple, usually unilateral macules of the face or extremities
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administered for 10 days has been prescribed. In one study, the wound grew
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rare progressive harmartomatous malformation involving the deeper veins
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reticulated vascular lesions, such as those in the following table:
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generalized or localized reticulated cutaneous vascular network.
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Livedo reticularis associated with collagen-vascular disease
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Cutaneous lesions described in patients with CMTC include
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It should not be confused with the more general term "
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275:dilated veins and venous lakes.
244:Nevus flammeus (port-wine stain)
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228:Neonatal lupus erythematosus
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83:Van Lohuizen syndrome
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310:mental retardation
125:café-au-lait spots
103:Signs and symptoms
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79:livedo reticularis
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391:Cato van Lohuizen
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764:Categories
697:DiseasesDB
397:References
344:gentamicin
316:ulceration
156:observed.
117:hemangioma
732:eMedicine
356:Prognosis
340:oxacillin
326:Treatment
270:Histology
188:Diagnosis
50:Specialty
743:Orphanet
737:derm/793
455:omim.org
431:cite web
421:28 April
314:Chronic
295:Glaucoma
204:Diseases
163:period.
161:neonatal
145:erythema
691:C536226
670:: Q82.8
460:10 July
726:001478
680:219250
385:Eponym
167:Causes
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702:33782
748:1556
686:MeSH
675:OMIM
462:2017
437:link
423:2019
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