Weill-Marchesani syndrome comprises short stature, brachydactyly, 
microspherophakia, glaucoma, and ectopia lentis is regarded as an autosomal 
recessive trait (McKusick 277600). We present two families each with affected 
individuals in 3 generations demonstrating autosomal dominant inheritance of 
Weill-Marchesani syndrome. Linkage analysis in these 2 families suggests a gene 
for Weill-Marchesani syndrome maps to 15q21.1. The dislocated lenses and 
connective tissue disorder in these families suggests that fibrillin-1 and 
microfibril-associated protein 1, which both map to 15q21.1, are candidate genes 
for Weill-Marchesani syndrome. Immunohistochemistry staining of skin sections 
from family 1 showed an apparent decrease in fibrillin staining compared to 
control individuals.