Angiodysplastic syndrome with capillary and venous malformation associated with soft tissue hypotrophy.
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.
Bircher
A J
AJ
Department of Dermatology, University of Basel, Switzerland.
Koo
J Y
JY
Frieden
I J
IJ
Berger
T G
TG
D000293:Adolescent; D000328:Adult; D016888:Angiodysplasia / Q000150:complications / Q000473:pathology*; D001284:Atrophy; D002196:Capillaries / Q000002:abnormalities; D002648:Child; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007715:Klippel-Trenaunay-Weber Syndrome / Q000473:pathology*; D007866:Leg / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D017445:Skin Diseases, Vascular / Q000150:complications / Q000473:pathology*; D013577:Syndrome