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Léri's Pleonosteosis A Study of a Family with a Review of the Literature
John G. Rukavina; Harold F. Falls; John F. Holt; Walter D. Block
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Departments of Dermatology and Syphilology, Ophthalmology, and Radiology, University of Michigan, Ann Arbor
1959 by The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 1959 Apr 01;41(3):397-408
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Abstract

A study of a family in which features of a systemic disease were exhibited consistent with the diagnosis of Léri's pleonosteosis has been presented. It is believed to be the first North American family to be reported. The findings are those of a widespread constitutional disease which involves chiefly the connectivetissue system. The syndrome appears to be the consequence of a gene exhibiting an autosomal dominant mode of transmission. The gene presents good penetrance and a considerable variance in its range of expressivity. Certain ophthalmological, cutaneous, osseous, and roentgenographic findings present in the reported family and mentioned in the literature are considered as possibly being additional expressions of this pleiotropic syndrome. More necropsy material is needed with which to understand this important syndrome better.

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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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