We report on a female aged 13 years, whose scalp hair began to disappe
ar at the age of 9 years, leaving only sparse wispy hairs in the parie
tal-occipital region. Eyelashes, eyebrows and body hair were unaffecte
d. There were no signs of ectodermal dysplasia on the skin, nails, tee
th and eyes nor other congenital abnormalities. The family pedigree sh
owed 15 relatives similarly affected according to an autosomal dominan
t mode of transmission. Clinical, genetic, histological and ultrastruc
tural aspects led to a diagnosis of hereditary hypotrichosis simplex o
f the scalp (Toribio-Quinones type).