Congenital absence or unusual patterns of human dermatoglyphs (fingerp
rints) occur in several syndromes that are rare and poorly understood.
The abnormalities of dermatoglyphs fall into four categories: complet
e absence, ridge-hypoplasia, ridge dissociation, and ridges-off-the-en
d. Complete congenital absence of ridges is an exceedingly rare syndro
me that consists of neonatal blisters and milia, adult traumatic blist
ering and fissuring, absence of sweating, contracture of digits, and a
bsence of dermatoglyphs on the hands and feet. The syndrome is inherit
ed in an autosomal dominant pattern, and only two kindreds have been d
escribed in the literature. We describe a newly identified patient and
kindred with findings similar to the previously reported cases and re
view the clinical and histopathologic findings of this syndrome.