Background. Pseudopelade is a rare idiopathic progressive alopecia tha
t has no initial inflammatory phase. Even so, pseudopelade has been cl
assified as a scarring alopecia with postinflammatory alopecias. Atrop
hy rather than true scarring is the cardinal feature of pseudopelade.
Materials and Methods. A review of reported cases of pseudopelade, esp
ecially those with a family history, was undertaken. The discovery of
a young man and his mother, who had pseudopelade, plus two other famil
ial cases prompted this study. Results. Examination of the clinical an
d pathologic material shows pseudopelade to be a disease separate from
other alopecias. The striking familial association in these cases dem
onstrates a plausible etiology. Conclusions. Pseudopelade is an autoso
mal dominant disorder of the scalp marked by atrophic loss of hair fol
licles.