Pachydermodactyly is a rare form of digital fibromatosis. There are on
ly 15 cases reported in the literature, all with a negative family his
tory. Only one of the previously reported cases was a woman. We now re
port 2 female cases, mother and daughter, who, to the best of our know
ledge, are the first patients with familial pachydermodactyly. The his
tological evolution of this disease over the years had not been previo
usly reported.