本邦における基底細胞母はん症候群についての検討　　自験例５例を含めて

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  • Basal cell nevus syndrome: Report of five cases with a review of the literatures in Japan

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Basal Cell Nevus Syndrome (BCNS) is primarily considered an autosomal dominant hereditary disease with the basal cell epithelioma, multiple jaw cysts and various skin and skeletal anomalies since Gorlin and Goltz reported the idea of this syndrome in 1960.<BR>The cases reported as BCNS for the past two decades, however, showed several differences in symptoms from the original idea, such as a lack of skin lesion or jaw cyst.<BR>The present study is intended to find a rationale of the diagnostic cues to BCNS by reviewing 60 cases reported in Japan involving 5 cases experienced by us.<BR>The result indicates that BCNS could be diagnosed based on the condition of either skin lesion or multiple jaw cysts with one of the following symptoms: Evidence of a family history, bifid ribs, pits, lamellar calcification of the falx cerebri and other skeletal anomalies. It is considered that these criteria are pertinent to those reported by Rayner (1976) and Gundlach (1979).

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