Glaessl A, Hohenlautner U, Landthaler M, Vogt T
Department of Dermatology, University of Regensburg, Regensburg, Germany.
Dermatol Surg. 2000 Feb;26(2):152-4. doi: 10.1046/j.1524-4725.2000.99146.x.
We present the case of a 32-year-old woman with a large recurrent multifocal basal cell carcinoma on the scalp. Conspicuous accompanying symptoms were multiple periorbital milia, hypotrichosis of the body and the scalp, and hypohidrosis. The sparse hair of the scalp showed further abnormalities such as pili torti, as well as flattened, irregularly curly hairs.
In 1964, Bazex et al. described a syndrome characterized by congenital hypotrichosis, follicular atrophoderma, and basocellular neoplasms that included basal cell nevi and early onset basal cell carcinomas. The Bazex-Dupré-Christol syndrome is a rare X-linked dominant disease. A sporadic occurrence with the typical constellation of these symptoms has not yet been reported. The lack of a positive family history and no signs of follicular atrophoderma argues for a sporadic occurrence of a Bazex-Dupré-Christol-like syndrome. The case reported shares several features with the classic Bazex-Dupré-Christol syndrome.
Our report documents the necessity to look for early development of basal cell carcinomas in patients who show signs of the epidermal malformations described.
我们报告了一名32岁女性病例，其头皮患有巨大复发性多灶性基底细胞癌。明显的伴随症状包括多发性眶周粟丘疹、身体和头皮毛发稀少以及少汗症。头皮稀疏的头发还表现出其他异常，如扭曲发，以及扁平、不规则卷曲的毛发。
1964年，巴泽克斯等人描述了一种综合征，其特征为先天性毛发稀少、毛囊性皮肤萎缩以及基底细胞肿瘤，包括基底细胞痣和早发性基底细胞癌。巴泽克斯-迪普雷-克里斯托尔综合征是一种罕见的X连锁显性疾病。尚未有关于这些症状典型组合的散发病例报道。缺乏阳性家族史且无毛囊性皮肤萎缩迹象，提示可能为散发性巴泽克斯-迪普雷-克里斯托尔样综合征。所报告的病例与经典的巴泽克斯-迪普雷-克里斯托尔综合征有若干共同特征。
我们的报告证明了对于出现所述表皮畸形迹象的患者，有必要关注基底细胞癌的早期发展。
