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KMID : 0363219950330010166
Korean Journal of Dermatology
1995 Volume.33 No. 1 p.166 ~ p.171
Two Cases of Cutis Marmorata Telangietasia Congenita



Abstract
Cutis marmorata telangiectatica congenita (CMTC) was first described by Van Lohuizen in 1992 Cases of CMTC were reported later under various names, including Van Lohuizen's syndrome, congenital generalized phlebectasia, and reticulate vascular
nevus. It
is charaterized by persistent livedo reticularis, telangectasia and phlectasia from birth, and atrophy and superficial ulceration of the overlying skn are uncommonly encountered. They have a steady improvement with time, however various
congenital
abnormalties, such as varicosities, hemangiomas, hypoplasia or hypertrophy of soft tissue and bones, rarely generalized congenital fibromatosis, rectal and genital anomalies may be associated in approximately 50 percent of patients.
In case 1, the patient was 45-day-old female infant with reticulated mottling and atrophy of the skin on the right leg. In case 2, the patient was 30-day-old male infant with depressed, bluish, and reticulated patches on the both upper arms,
right
side
trunk, buttock and lower leg. Decreased circumferences of the affected extremities was observed in case 2. No other associated anomalies were found in physical and X-ray examinations in both cases. The skin biopsy of both had similar findings,
which
revealed mild dilatation and proliferation of capillaries, mild inflammatory cell infiltration and extravasated red blood cells in the dermis. On the clinical follow0up, almost all of the lesions disappeared spontanously in 1 year at case 1, and
some of
lesions also disappeared in 3 months in case 2. In addition, we reviewed and summerized the reported cases in Korea.(Kor J Dermatol 1995 ; 33(1) : 166~171)
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