Nevoid Basal Cell Carcinoma Syndrome の1例 A Case of Nevoid Basal Cell Carcinoma Syndrome





47歳女性のnevoid basal cell carcinoma syndrome(以下NBCCS)の1例を報告する。NBCCSは多発性または若年性の基底細胞癌,顎骨の多発性嚢胞(歯原性角化嚢胞),掌蹠のpits,異所性石灰化,骨格異常を含む多彩な臨床像を呈する常染色体優性遺伝母斑症であり,その原因遺伝子は9q22.3に位置するpatched geneと強い相同性を示す。自験例では全身に散在する36個の色素性病変を切除し,その内の11個が基底細胞癌(basal cell carcinoma,以下BCC)であり,一般のBCCに比較し,非露出部の発症が多く,組織学的には表在型が多かった。

We herein report the case of a 47-year-old Japanese woman who presented with nevoid basal cell carcinoma syndrome (NBCCS). The patient was characterized by multiple basal cell carcinomas (BCCs) of the skin, tiny palmoplantar pits, dental cysts and abnormalities in the bone. Based on Evans' criteria, the patient was diagnosed to have NBCCS. Eleven BCCs were excized: Five of them were found in unexposed areas while seven of them histologically revealed superficial type, thus suggesting that BCCs in unexposed areas and superficial BCCs are more common in NBCCS than sporadic BCCs.


  • 西日本皮膚科 = The Nishinihon journal of dermatology

    西日本皮膚科 = The Nishinihon journal of dermatology 64(2), 178-181, 2002-04-01

    Western Division of Japanese Dermatological Association

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