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先天性平滑肌错构瘤。15例患者的患病率、临床特征及随访情况

Congenital smooth muscle hamartoma. Prevalence, clinical findings, and follow-up in 15 patients.

作者信息

Zvulunov A, Rotem A, Merlob P, Metzker A

机构信息

Pediatric Dermatology Unit, Beilinson Medical Center, Petah Tiqwa, Israel.

出版信息

Am J Dis Child. 1990 Jul;144(7):782-4. doi: 10.1001/archpedi.1990.02150310050024.

DOI:10.1001/archpedi.1990.02150310050024
PMID:2356798
Abstract

Cogenital smooth muscle hamartoma is a congenital skin lesion characterized by proliferation of bundles of smooth muscle within the reticular dermis. We report on a group of 15 children with this lesion, confirmed by skin biopsy specimen, its prevalence, the occurrence of associated anomalies, and long- term follow-up. To our knowledge, this is the largest group of patients with this lesion described in the literature. The estimated prevalence is about 1:2600 live births with slight male predominance. The lesion is most frequent in the lumbosacral area (67% [10/15]) and a positive pseudo-Darier's sign is present in 80% (12/15) of patients. During the follow-up period of up to 7 years, the lesions enlarged slightly but became less prominent. No malignant transformation was observed.

摘要

先天性平滑肌瘤是一种先天性皮肤病变,其特征是网状真皮内平滑肌束增生。我们报告了一组经皮肤活检标本确诊的15例患有该病变的儿童,包括其患病率、相关异常的发生情况以及长期随访结果。据我们所知,这是文献中描述的患有该病变的最大患者群体。估计患病率约为1:2600活产,男性略占优势。该病变最常见于腰骶部区域(67%[10/15]),80%(12/15)的患者存在阳性假达里埃氏征。在长达7年的随访期间,病变略有增大,但变得不那么明显。未观察到恶性转化。

相似文献

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Congenital smooth muscle hamartoma. Prevalence, clinical findings, and follow-up in 15 patients.先天性平滑肌错构瘤。15例患者的患病率、临床特征及随访情况
Am J Dis Child. 1990 Jul;144(7):782-4. doi: 10.1001/archpedi.1990.02150310050024.
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Congenital smooth muscle hamartoma of the skin.先天性皮肤平滑肌错构瘤
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Congenital smooth-muscle hamartoma.先天性平滑肌错构瘤。
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Myokymia as a presenting sign of congenital smooth muscle hamartoma.肌束震颤作为先天性平滑肌错构瘤的首发体征。
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