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KMID : 0363220200580030168
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Korean Journal of Dermatology
2020 Volume.58 No. 3 p.168 ~ p.173
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Clinical Features and Natural Course of Cutis Marmorata Telangiectatica Congenita: A Single-center Retrospective Study of 34 Patients
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Lee Han-Jae
Park Jong-Seo
Kim Ji-Won
Kim Kyu-Han
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Abstract
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Background: Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital cutaneous anomaly characterized by persistent reticular skin lesion with a blue to purple color change. While the natural prognosis of the disease is generally considered good, limited data are available regarding the natural course of Korean patients with CMTC.
Objective: To investigate the clinical features and natural course of Korean patients with CMTC.
Methods: We performed a single-center retrospective study of 34 Korean patients diagnosed with CMTC at our institution between January 1999 and June 2019.
Results: A slight female predominance was observed (male:female ratio, 1：1.4) and 82.4% of the patients were diagnosed before 2 years of age. Body asymmetry (32.4%) and other vascular anomalies (14.7%) were the two anomalies most frequently associated with CMTC. Most of the skin lesions developed on the lower extremities (67.6% lower extremity only, 20.6% lower extremity and other body regions) and 85.3% of the lesions occurred unilaterally. Among 25 patients with one or more follow-up evaluations, 20 (80.0%) showed spontaneous fading of the skin lesion. However, none showed a complete resolution. Finally, statistical analysis did not reveal any significant variable associated with the natural prognosis of CMTC.
Conclusion: Korean patients with CMTC had similar clinical features and natural course as those described in the previous literature. Notably, a greater portion of the patients showed improvement in skin lesions compared to those in previous studies. However, complete resolution of the skin lesion seems to be rare, if not impossible.
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KEYWORD
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Cutis marmorata telangiectatica congenita, Prognosis
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