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1 型肌强直性营养不良患者的发育不良痣、皮肤黑素瘤和其他皮肤肿瘤:一项横断面研究。

Dysplastic nevi, cutaneous melanoma, and other skin neoplasms in patients with myotonic dystrophy type 1: a cross-sectional study.

机构信息

Department of Dermatology, Policlinico A. Gemelli, Università Cattolica del Sacro Cuore, Rome, Italy; Department of Dermatology, Barts NHS (National Health System) Trust, London, United Kingdom.

Department of Neurology, Policlinico A. Gemelli, Università Cattolica del Sacro Cuore, Rome, Italy.

出版信息

J Am Acad Dermatol. 2015 Jan;72(1):85-91. doi: 10.1016/j.jaad.2014.09.038. Epub 2014 Nov 5.

DOI:10.1016/j.jaad.2014.09.038
PMID:25440959
Abstract

BACKGROUND

Myotonic dystrophy type 1 (MD1) is reported to be associated with internal malignancies. The association of myotonic dystrophy with cutaneous tumors is not fully understood.

OBJECTIVE

We sought to explore the total nevi count and the presence of atypical nevi, cutaneous melanoma, and other skin neoplasms in a representative cohort of patients with MD1 and to compare the findings with age- and sex-matched control subjects.

METHODS

In all, 90 patients with MD1 and 103 age- and sex-matched control subjects were assessed for cutaneous neoplasms by clinical skin and epiluminescence examination (dermoscopy). Where indicated, subsequent excisions were performed. In patients with MD1, leukocyte n(CTG) expansion was measured.

RESULTS

Patients with MD1 showed significantly higher numbers of nevi, dysplastic nevi, and melanomas despite a significantly greater proportion of the control subjects reporting sunburns. In addition, we found a significantly greater number of pilomatrixoma in patients with MD1.

LIMITATIONS

Our study is limited by the fact that there is no agreed-upon standardized technique to assess for prior sun exposure. Further research in the association of cutaneous neoplasms and MD1 including vitamin D and molecular biological techniques are also recommended.

CONCLUSION

MD1 itself may predispose to development of skin tumors.

摘要

背景

肌强直性营养不良 1 型(MD1)据报道与内部恶性肿瘤有关。肌强直性营养不良与皮肤肿瘤的关联尚未完全阐明。

目的

我们旨在探讨在代表性 MD1 患者队列中总痣计数和不典型痣、皮肤黑色素瘤和其他皮肤肿瘤的存在情况,并将这些发现与年龄和性别匹配的对照组进行比较。

方法

通过临床皮肤和荧光检查(皮肤镜检查)对 90 名 MD1 患者和 103 名年龄和性别匹配的对照组进行皮肤肿瘤评估。如有需要,进行后续切除。在 MD1 患者中,测量白细胞 n(CTG)扩张。

结果

尽管对照组中有更多的人报告晒伤,但 MD1 患者的痣、发育不良痣和黑色素瘤数量明显更多。此外,我们发现 MD1 患者的毛母细胞瘤数量明显更多。

局限性

我们的研究受到限制,因为目前没有用于评估既往日晒的公认标准化技术。还建议对皮肤肿瘤与 MD1 的关联进行包括维生素 D 和分子生物学技术在内的进一步研究。

结论

MD1 本身可能易患皮肤肿瘤。

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