Léri's syndrome I
- Léri's pleonosteosis syndrome
A form of congenital physical malformation characterised mainly by osseous abnormalities due to precocious and excessive ossification of the bones of cartilaginous origin associated with enlargement of the epiphyses. There is generalised limitation of joint mobility and contractures due to thickening of fingers and toes. Cutaneous changes consist mainly of hollow palms with thickened palmar and forearm fasciae, accentuated skin creases between thick palmar pads, and nodules. Usually associated with mongoloid facies, shuffling gait, and short stature. Normal mental development. Both sexes affected; onset in early infancy. Etiology unknown. Inheritance is autosomal dominant.
- A. Léri:
Une maladie congénitale et héréditaire de l'ossification: la pléonostéose familiale.
Bulletins et memoires de la Société medicale des hôpitaux de Paris, 1921, 3 sér., 45: 1228-1230. Une dystrophie osseuse géneralisée congénitale et hereditaire; la pléonosteose familiale.
La presse médicale, Paris, 1922, 30: 13-16.