Dermatologic care of patients with differences of sex development.
Dermatologic care of patients with differences of sex development.
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DOI：
10.1097/jw9.0000000000000106
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发表时间：
2023-10
影响因子：
--
通讯作者：
Yeung, Howa
中科院分区：
文献类型：
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作者：
Gold, Sarah;Huang, Christina;Radi, Rakan;Gupta, Pranav;Felner, Eric I;Haw, Jeehea Sonya;Childress, Krista;Sokkary, Nancy;Tangpricha, Vin;Goodman, Michael;Yeung, Howa
性发育差异（DSD 或性发育障碍）是罕见的先天性疾病，其特征是染色体、性腺或解剖性别发育不典型。皮肤科护理是 DSD 患者所需的多学科护理的重要组成部分。本文讨论了 DSD 最常见的原发性皮肤病表现以及 DSD 患者激素和手术治疗的皮肤表现。在 PubMed 上发表文章，包括病例系列和病例报告。选择是通过与多学科专家团队审查现有文献来进行的。叙事回顾。本文并非系统综述。在克兰费尔特综合征中，已描述了难治性腿部溃疡和色素失禁。特纳综合征与淋巴管畸形、晕痣、皮炎和牛皮癣有关。男性化可见于某些形式的先天性肾上腺增生症，其中痤疮和多毛症很常见。皮肤科医生应根据妊娠潜力考虑 DSD 皮肤病治疗的致畸风险。睾酮替代疗法通常用于治疗克氏综合征、雄激素不敏感综合征、5-α还原酶缺乏症、性腺发育不全或卵睾 DSD，可能会导致痤疮。
Differences of sex development (DSD or disorders of sex development) are uncommon congenital conditions, characterized by atypical development of chromosomal, gonadal, or anatomic sex. Dermatologic care is an important component of the multidisciplinary care needed for individuals with DSD. This article discusses the most common primary dermatologic manifestations of DSD in addition to the cutaneous manifestations of hormonal and surgical therapies in individuals with DSD. Published articles including case series and case reports on PubMed. Selection was conducted by examining existing literature with a team of multidisciplinary specialists. Narrative review. This article was not conducted as a systematic review. In Klinefelter syndrome, refractory leg ulcers and incontinentia pigmenti have been described. Turner syndrome is associated with lymphatic malformations, halo nevi, dermatitis, and psoriasis. Virilization can be seen in some forms of congenital adrenal hyperplasia, where acne and hirsutism are common. Dermatologists should consider teratogenic risk for treatments of skin conditions in DSD depending on pregnancy potential. Testosterone replacement, commonly used for Klinefelter syndrome, androgen insensitivity syndrome, 5-alpha reductase deficiency, gonadal dysgenesis, or ovotesticular DSD, may cause acne.