Background: Persons with ectodermal dysplasias classically have defects in
hair, teeth, nails, and sweat glands. Other tissues derived from ectoderm m
ay also be involved. Ocular involvement in ectodermal dysplasias primarily
occurs in anhidrotic forms. Methods: We describe a father and son with hidr
otic ectodermal dysplasia. Results: Both patients had recurrent corneal epi
thelial defects from birth, corneal neovascularization, and strabismus. The
father had cataracts with crystalline and amorphous inclusions at an early
age. Both patients also had alopecia and skin abnormalities. Conclusions:
A father and son with a previously unreported hidrotic ectodermal dysplasia
and unusual corneal findings are described.