We describe a total of 18 individuals, in 3 families, with a median no
dule of the upper lip. In family 1, the proposita, an 8-month-old infa
nt girl, was otherwise phenotypically normal except for a median nodul
e of the upper lip. The proposita's elder brother and mother, both phe
notypically normal, also had the similar nodule of the upper lip. On t
he mother's side, the proposita's greatgrandmother, greatgrandaunt, gr
andfather, greataunt, two aunts, and one female cousin all had a media
n nodule of the upper lip. In family 2, the proposita, proposita's mot
her and maternal grandfather had a median nodule of the upper lip. In
family 3, the proposita, proposita's father, paternal grandfather, pat
ernal uncle, and cousin had a median nodule of the upper lip. Analysis
of 3 families indicates that the condition is an autosomal dominant t
rait. (C) 1994 Wiley-Liss, Inc.