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先天性大理石样皮肤毛细血管扩张症:9例报告及文献复习

Cutis marmorata telangiectatica congenita: report of nine cases and review of the literature.

作者信息

Powell S T, Su W P

出版信息

Cutis. 1984 Sep;34(3):305-12.

PMID:6386356
Abstract

The major clinical features of nine patients (seven women and two men) with cutis marmorata telangiectatica congenita (CMTC) were persistent cutis marmorata, spider nevus-like telangiectasia, and phlebectasia. Three of the patients had persistent ulcers and atrophy. In two patients, atrophie blanche-like lesions developed on the legs with ulcers. No patient had a family history of a similar disorder. Skin lesions were present at birth in seven cases, at the age of eight months in one case, and at eighteen months in one case. Three patients had clinical improvement of the skin lesions with age. Eight of the nine patients had associated physical anomalies. Biopsy specimens showed an increase in the number and size of the blood vessels in the upper dermis. Alkaline phosphatase staining gave negative results in the dilated blood vessels of the single case in which this histochemical study was done.

摘要

9例先天性大理石样皮肤毛细血管扩张症(CMTC)患者(7名女性和2名男性)的主要临床特征为持续性大理石样皮肤、蜘蛛痣样毛细血管扩张和静脉扩张。3例患者有持续性溃疡和萎缩。2例患者腿部出现类白色萎缩性病变并伴有溃疡。所有患者均无类似疾病家族史。7例患者出生时即有皮肤病变,1例在8个月大时出现,1例在18个月大时出现。3例患者的皮肤病变随年龄增长有临床改善。9例患者中有8例伴有身体异常。活检标本显示真皮上层血管数量和大小增加。在进行此项组织化学研究的唯一病例中,碱性磷酸酶染色在扩张血管中呈阴性结果。

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