Manifestations of the condition are generally limited to
the skin. Wrinkling or puckering of the skin at the temple region (original
description noted similarity to obstetric “forceps marks”). Occasionally,
guttate areas cover middle forehead and chin. Usually, the affection is
bilateral, and only two of the original 38 patients described by Brauer had
a unilateral occurrence. One case report of a 2-month-old child suggested a
possible association between the syndrome and tetralogy of Fallot, although
no other related cardiac anomalies have been described. Brauer and Setleis
(focal facial dermal dysplasia type II) syndromes have been suggested by some authors to be
the same condition.