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선천모세혈관확장대리석피부의 임상 양상과 자연 경과: 후향적 단일기관 연구

Clinical Features and Natural Course of Cutis Marmorata Telangiectatica Congenita: A Single-center Retrospective Study of 34 Patients

대한피부과학회지 2020년 58권 3호 p.168 ~ 173
이한재 ( Lee Han-Jae ) - 서울대학교 의과대학 피부과학교실

박종서 ( Park Jong-Seo ) - 서울대학교 의과대학 피부과학교실
김지원 ( Kim Ji-Won ) - 서울대학교 의과대학 피부과학교실
김규한 ( Kim Kyu-Han ) - 서울대학교 의과대학 피부과학교실

Abstract


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.

키워드

Cutis marmorata telangiectatica congenita; Prognosis
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