Papillon-lefevre syndrome (hyperkeratosis palmoplantaris with periodontosis)

General: Autosomal recessive; o 717h75h nset between ages 1 and 4 years.

Ocular: Nystagmus.

Clinical: Hyperkeratosis of the palms and soles associated with destruction of the periodontal ligament and premature exfoliation of the teeth; bad breath; loose teeth; loss of teeth.

Mhaiskar D, et al. Papillon-Lefevre syndromes with ocular nystagmus (a case report). J Postgrad Med

Papillon MM, Lefevre P. Deux Cas de Keratodermie Palmaire et taire Symetrique Familiale (Maladie de Meleda) Chez le Frere et la Soeur. Coexistence dans les Deux Cas d' Alterations Dentaires Graves. Bull Soc Fr Dermatol Syphil 1924; 31:82-87.