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巴泽克斯综合征:副肿瘤性肢端角化病。

Bazex syndrome: acrokeratosis paraneoplastica.

作者信息

Bolognia J L

机构信息

Department of Dermatology, Yale University School of Medicine, New Haven, CT 06520, USA.

出版信息

Semin Dermatol. 1995 Jun;14(2):84-9. doi: 10.1016/s1085-5629(05)80002-1.

DOI:10.1016/s1085-5629(05)80002-1
PMID:7640201
Abstract

The focus of this article is acrokeratosis paraneoplastica, one of two disorders that have acquired the eponym Bazex syndrome. To date, all of the patients reported in the literature have had an underlying neoplasm, most commonly squamous cell carcinoma of the upper aerodigestive tract. In this review of 113 cases of acrokeratosis paraneoplastica (mean age, 61 years; 105 males, 8 females), the psoriasiform lesions preceded the diagnosis of the associated malignancy in 73 (67%) of 109 patients, whereas the cutaneous manifestations followed the diagnosis of the neoplasm in only 16 (15%) of 109; in the remainder, the onset of the skin lesions and the diagnosis of the tumor occurred simultaneously. Therefore, awareness of the cutaneous signs of Bazex syndrome is of obvious importance to dermatologists. Evidence in favor of the paraneoplastic nature of this disease is as follows: in 81 (93%) of 87 patients with adequate clinical descriptions, the skin lesions either improved significantly (or resolved) when the underlying neoplasm was treated or they remained unchanged in the setting of persistent disease. Occasionally, the reappearance of skin lesions has signaled a recurrence of the tumor.

摘要

本文的重点是副肿瘤性肢端角化病,它是获得了巴泽克斯综合征这一名称的两种疾病之一。迄今为止,文献报道的所有患者都有潜在的肿瘤,最常见的是上呼吸消化道鳞状细胞癌。在这篇对113例副肿瘤性肢端角化病患者(平均年龄61岁;男性105例,女性8例)的综述中,109例患者中有73例(67%)的银屑病样皮损先于相关恶性肿瘤的诊断出现,而在109例中只有16例(15%)的皮肤表现出现在肿瘤诊断之后;其余患者中,皮肤病变的出现与肿瘤的诊断同时发生。因此,对皮肤科医生来说,了解巴泽克斯综合征的皮肤体征显然很重要。支持这种疾病具有副肿瘤性质的证据如下:在87例有充分临床描述的患者中,有81例(93%)在治疗潜在肿瘤时皮肤病变明显改善(或消退),或者在疾病持续存在的情况下保持不变。偶尔,皮肤病变的再次出现预示着肿瘤复发。

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[Paraneoplasms of the skin].
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