Bircher A J, Koo J Y, Frieden I J, Berger T G
Department of Dermatology, University of Basel, Switzerland.
Dermatology. 1994;189(3):292-6. doi: 10.1159/000246865.
Angiodysplastic syndromes include a vascular malformation which may often be associated with secondary changes such as further vascular abnormalities, soft tissue and bone hypertrophy. One of the best known is the syndrome triad originally described by Klippel and Trenaunay, which includes a unilateral capillary malformation, ectatic veins and osseous and soft tissue hypertrophy. A female patient is reported who had an extensive capillary malformation, discrete ectatic veins overlying an extensive venous malformation and soft tissue hypotrophy without bone involvement. Our patient and cases from the literature illustrate the occurrence of 'atypical', hypotrophic variants of angiodysplastic syndromes. Discrete superficial angiodysplasias may overlie deep, extensive malformations, and identification of such cases is important with regard to management and prognosis. The current classification of angiodysplasias is based on the primary vascular malformation. The use of eponyms should be avoided, because they do not contribute to the diagnosis and management of such cases.
血管发育异常综合征包括一种血管畸形,其常与继发性改变相关,如进一步的血管异常、软组织和骨肥大。最著名的之一是最初由克-特二氏描述的综合征三联征,包括单侧毛细血管畸形、扩张静脉以及骨和软组织肥大。本文报告了一名女性患者,她有广泛的毛细血管畸形、覆盖广泛静脉畸形的离散扩张静脉以及无骨受累的软组织萎缩。我们的患者以及文献中的病例说明了血管发育异常综合征“非典型”、萎缩性变体的发生情况。离散的浅表血管发育异常可能覆盖深部广泛的畸形,识别此类病例对于管理和预后很重要。目前血管发育异常的分类基于原发性血管畸形。应避免使用人名命名法,因为它们无助于此类病例的诊断和管理。