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眼皮肤白化病伴发育异常痣综合征及多发原发性无色素性黑色素瘤

Dysplastic nevus syndrome with multiple primary amelanotic melanomas in oculocutaneous albinism.

作者信息

Pehamberger H, Hönigsmann H, Wolff K

出版信息

J Am Acad Dermatol. 1984 Oct;11(4 Pt 2):731-5. doi: 10.1016/s0190-9622(84)70230-1.

DOI:10.1016/s0190-9622(84)70230-1
PMID:6490997
Abstract

Melanomas are rare in albinos, although the incidence of solar radiation-induced skin tumors is extremely high because of the absence of photoprotective melanin. This report describes a 40-year-old white woman with tyrosinase-negative oculocutaneous albinism who developed four primary amelanotic melanomas--three of the superficial spreading and one of the nodular type-and, in addition, displayed nevi that exhibited histologically the characteristic features of dysplastic nevi. The concomitant occurrence of multiple primary melanomas and several dysplastic nevi classifies the patient's condition as "dysplastic nevus syndrome," which to our knowledge has not been described in albinism so far.

摘要

黑色素瘤在白化病患者中较为罕见,尽管由于缺乏光保护黑色素,太阳辐射诱发的皮肤肿瘤发病率极高。本报告描述了一名40岁的白人女性,患有酪氨酸酶阴性的眼皮肤白化病,她患上了4例原发性无色素性黑色素瘤——3例为浅表扩散型,1例为结节型——此外,还出现了组织学上表现为发育异常痣特征的痣。多发性原发性黑色素瘤和数例发育异常痣同时出现,将该患者的病情归类为“发育异常痣综合征”,据我们所知,迄今为止在白化病中尚未有过此类描述。

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Dysplastic nevus syndrome with multiple primary amelanotic melanomas in oculocutaneous albinism.眼皮肤白化病伴发育异常痣综合征及多发原发性无色素性黑色素瘤
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