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色素血管性斑痣性错构瘤病

Phakomatosis pigmentovascularis.

作者信息

Van Gysel D, Oranje A P, Stroink H, Simonsz H J

机构信息

Subdivision of Pediatric Dermatology, Sophia Children's Hospital/University Hospital, Rotterdam, The Netherlands.

出版信息

Pediatr Dermatol. 1996 Jan-Feb;13(1):33-5. doi: 10.1111/j.1525-1470.1996.tb01184.x.

DOI:10.1111/j.1525-1470.1996.tb01184.x
PMID:8919522
Abstract

We report a patient with phakomatosis pigmentovascularis IIb and numerous iris hamartomas. Phakomatosis pigmentovascularis IIb is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed.

摘要

我们报告了一名患有色素血管性斑痣性错构瘤病IIb型及大量虹膜错构瘤的患者。色素血管性斑痣性错构瘤病IIb型的特征是在同一个体中同时出现火焰状痣、蒙古斑,有时还有贫血痣,并伴有全身受累。据我们所知,与多个虹膜错构瘤的关联仅被报道过一次。这例第二位患者提示这种关联可能更为常见。更多的报告将表明这种关联是否比目前所认为的更为频繁。

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