Cutis marmorata telangiectatica congenita is characterized by reticular erythema that is either generalized over the entire body or localized to a specific area or. Cutis marmorata telangiectatica congenita (CMTC) is a rare condition that causes a marbled pattern on the skin. Learn the symptoms, other. Cutis marmorata telangiectatica congenita. Authoritative facts about the skin from DermNet New Zealand Trust.
|Published (Last):||24 October 2017|
|PDF File Size:||7.99 Mb|
|ePub File Size:||16.65 Mb|
|Price:||Free* [*Free Regsitration Required]|
Associated anomalies have been reported by many authors. Studies indicated the primary involvement of capillaries, venules and veins, and possibly also that of arterioles and lymphatics. The Galeazzi sign flexing the hips and knees while the patient is supine to reveal a discrepancy in femur length showed symmetrical knee height.
In conclusion, we believe that CMTC is a relatively mild condition. Blackwell Scientific Publications; For: No CMTC or other vascular lesions in the area around the eyes were observed in the other 2 cases. Cutis marmorata telangiectatica congenita CMTC is a congeniha localized or generalized vascular anomaly characterized by a persistent cutis marmorata pattern with a marbled bluish to deep purple appearance, spider nevus-like telangiectasia, phlebectasia and, occasionally, ulceration and atrophy of the affected skin.
Cutis marmorata telangiectatica congenita. Suffolk, VA Phone: The lesion did not disappear congenitq warming. During the follow-up, there was no visible neurological damage and the seizures did not return. There is no specific treatment for the skin lesions of cutis marmorata telangiectatica congenita.
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. What is cutis marmorata telangiectatica congenita CMTC? This article has been cited by other articles in PMC. Read clinical updates and the latest insights from Boston Children’s specialists. Moore CA, et al. One of them was the son of consanguineous parents, and the other had a mother with some of the same congenital abnormalities and a sister with slow motor development along with abnormal hair and, possibly, mild cutis marmorata.
Our experience in treating numerous patients with CMTC gives us the depth of knowledge about what symptoms to look for and how to plan treatment accordingly. Ocular abnormalities, particularly glaucoma, have been reported in patients with facial lesions associated with cutis marmorata telangiectatica congenita.
Clin Exp Dermatol ; She had granular retinal pigmentation, which remained stationary during the follow-up. Macrocephaly-cutis marmorata telangiectasia congenita: Cutis marmoratatelangeictaticacongenita Van Lohuizen syndrome.
Cutis marmorata telangiectatica congenita – Wikipedia
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. It should not be confused with cutis marmorataa normal physiologic skin mottling in cool environments.
CMTC is an uncommon, sporadic congenital vascular malformation characterized by a generalized or localized reticulated cutaneous vascular network.
Affected children with limb length discrepancies should be monitored with standing leg-length radiographs cngenita the age of 10 girls or 12 boysor if the limb length discrepancy is greater or equal to 2 cm. It should not be confused with the more general term ” cutis marmorata “, which refers to livedo reticularis caused by cold. Get free access to newly published articles. Other search option s Alphabetical list. The pathogenesis of CMTC is unknown. The cutaneous telangiectaitca most commonly occur on the legs, less commonly on the arms and trunk, and rarely involve the face and scalp.
Cutis Marmorata Telangiectatica Congeita (CMTC) | Boston Children’s Hospital
Since many cases of CMTC are mild and clear up without treatment, the disorder may be under-diagnosed making it difficult to determine the true frequency of CMTC in the general population. However, several members of a family may occasionally have CMTC. Standard ophthalmologic examination in all cases of CMTC seems unnecessary and should be limited to the cases in which there congneita vascular lesions around the eyes. However, many if not cuutis of those cases represent forms of Klippel-Trenaunay syndrome or related disorders, in particular Cowden’s disease.
Pediatr Dermatol ; In severe cases, an underlying defect of the bones of the skull may also be present. Important differences exist between the congenital condition and physiologic cutis marmorata that can help to distinguish between these lesions. Kienast and Hoeger 2 recently proposed a set of criteria for the diagnosis of cutis marmorata telangiectatica congenita Box 2. Request an Appointment Request a Second Opinion.
Amitai DB, et al. Support Center Support Center. Giuliano F, et al. It has been suggested that these 3 entities should be classified as a group of vascular diseases associated with other developmental defects representing defects of the mesodermal system during embryonic life.
Cutis marmorata may occur in conjunction with other syndromes but is not diagnostic. Etiology The etiology remains unknown. Possible associated anomalies should be excluded by a careful clinical examination. CMTC may appear indented due to dermal atrophy loss of dermis. It has a marbled bluish to deep-purple appearance.
One theory suggests that abnormal pericyte recruitment can cause skin capillaries to contract inappropriately. In 2 cases, the skin lesions initially appeared less reticulated and evolved into the typical CMTC lesions in the first few weeks of life.
Cutis Marmorata Telangiectatica Congeita (CMTC)
Diagnostic methods Diagnosis is clinical. Atrophy of the epidermis and rarely ulceration may also occur. The child with the retinal pigmentation showed CMTC lesions on the left side of his face.