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Léri-Joanny syndrome
Also known as:
Léri’s disease
Leri’s type of osteopetrhosis
Léri’s syndrome I
Associated persons:
Jean Paul Joanny
André Léri
Description:
Melorheostosis - candle bone disease – is a very rare disorder of bone characterised by striated sclerosing - irregular linear areas of increased density along the shafts of the long bones, due to an ostal or periostal osteosclerosis in normal mineral metabolism. Occurs in two forms, monostotic and polystotic, affecting single or multiple bones, respectively. If condition has an early onset, premature closure of epiphysis and dwarfism may result. Limb motion may be reduced, because of ankylosis or muscle involvement or both. In adults, x-ray findings show linear densities associated with areas of osteophytic periosteal excrescences resembling melted wax flowing down the side of a candle, hence the synonym melorheostosis Both sexes affected. Onset almost exclusively in early infancy, but onset in adult age has been reported. Etiology unknown.
Bibliography:
- A. Leri, J. P. Joanny:
Une affection non décriete des os: Hyperostose «en coulée» sur toute la longueur d’un membre ou “mélorhéostose”.
Bulletins et memoires de la Société medicale des hôpitaux de Paris, 1922, 46: 1141-1145.
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