Background. Pseudopelade is a rare idiopathic progressive alopecia that has no initial inflammatory phase. Even so, pseudopelade has been classified as a scarring alopecia with postinflammatory alopecias. Atrophy rather than true scarring is the cardinal feature of pseudopelade.
Materials and Methods. A review of reported cases of pseudopelade, especially those with a family history, was undertaken. The discovery of a young man and his mother, who had pseudopelade, plus two other familial cases prompted this study.
Results. Examination of the clinical and pathologic material shows pseudopelade to be a disease separate from other alopecias. The striking familial association in these cases demonstrates a plausible etiology.
Conclusions. Pseudopelade is an autosomal dominant disorder of the scalp marked by atrophic loss of hair follicles.