Uehlinger's Disease

概要

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Uehlinger's disease is rare, systemic bone disease which is characterized by hyperostosis, pachydermia and clubbing finger.Since Uehlinger reported the disease as hyperostosis generalisata with pachydermia in 1941, it has been reported in various name such as Familiäre acromegalie-ähnliche Erkrankung des Skelettes (Oehme 1919), Megalia ossium et cutis (Grönberg 1927), Cutis verticis gyrata-Acromegalie-Osteoperiostitis hyperplastica (Renander), Pachydermoperiostosis (Schwarby 1962).The first case is 23 years man, who suffered from pain and hydrops of bilateral knees since 19 years. The second case is 22 years man whose chief complaint is the deformity of fingers. These two cases showed typical pachydermia on the front, clubbing finger and hyperostosis on the diaphysis without any particular findings on laboratory examination. The third case is 45 years man with polyarthralgia. This is a little atypical, because he shows neither pachydermia nor periosteal thickening in the diaphysis.These cases have no abnormality in cardio-vascular, pulmonary or gastrointestinal system.Differential diagnosis and nomenclature was discussed.
西日本整形・災害外科学会の論文