Periodontosis, premature loss of primary and
adult teeth, hypodontia. Hyperkeratosis palmoplantaris with transgression
onto the elbows and the knees, as well as other areas, hypotrichosis, nail
fragility, ectopic intracranial (essentially dura mater) calcifications,
eyelid cysts. May be associated with mental retardation, deafness,
retardation of skeletal maturation, osteoporosis, arachnodactyly, and,
rarely, acroosteolysis. The periodontosis is associated with an elevated
rate of cutaneous abscesses and pyogenic infections of internal organs
(liver, spleen mainly). No constant association has been found with
immunologic disorders. Patients affected with autosomal dominant palmoplantar
keratosis often present with malignancies of the esophagus and/or other
malignancies (lymphomas, pancreas, breast, etc.). This feature may be associated with
severe stricture of the esophagus in childhood. In the absence of genetic
differential diagnosis, palmoplantar
keratosis must be considered. Aggressive dental treatment
and retinoid medication helps to keep definitive denture.