基底細胞母斑症候群と思われる 1 症例

概要

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The syndrome of multiple nevoid basal cell epithelioma, multiple odontogenic keratocyst and skeletal anomality is far more complex and encompassing than was supposed when first described by Jarisch in 1894. The change that involved skin includes multiple nevoid basal cell epithelioma appearing in childhood or at puberity, dyskeratosis, milias, cyst and fibromas or neurofibromas or both, especially the extremites. The cause of the syndrome is unknown and autosomal dominant inheritance which is determined by a highly penetrance gene and multiple variable effects. There is no predilection on the basis of sex. A 12-year-old girl had many features of the basal cell nevus syndrome which were frontal bossing, wide nasal root, hyperterorism, deep-set eyes, several bifid ribs, calcification of the falx cerbri. Long term observation will be necessary to detect the possible development of either basal cell epithelioma or the recurrence of the odontogenic keratocyst.
九州歯科学会の論文
1984-02-25