18三体伴广泛的皮肤钙质沉着症。

Q3 Medicine
Skin health and disease Pub Date : 2025-04-22 eCollection Date: 2025-06-01 DOI:10.1093/skinhd/vzaf023
Airin Sato, Yu Matsui, Teruhiko Makino, Tadamichi Shimizu
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引用次数: 0

摘要

18三体是第二常见的常染色体三体,与高死亡率相关,只有5-10%的受影响个体存活过一岁。因此,长期幸存者的合并症很少被报道。我们描述的情况下,6岁的东亚女孩与三体18谁提出了皮肤钙质沉着症。患者最初在左手腕出现一个坚硬、无压痛的皮下结节,后来扩散到前臂和上臂。体格检查和影像学显示其他四肢有广泛的皮下结节。高频超声显示低回声肿块伴后侧声影,皮肤活检显示脂肪坏死及钙沉积。钙、磷、甲状旁腺激素或维生素d水平未检测到明显异常。根据实验室检查结果和患者的病史,不太可能是皮肤钙质沉着症的代谢、营养、炎症和医源性原因。因此,这种情况被归类为营养不良或特发性皮肤钙质沉着症。该患者作为门诊病人进行治疗,没有特殊治疗。本文讨论皮肤钙质沉着症的发病机制及其与18三体相关性研究有限的原因。据我们所知,这是第一个将皮肤钙质沉着症描述为小儿18三体患者的伴随疾病的报告,预计对这种疾病的认识的提高可能会导致未来报告病例的增加。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Trisomy 18 with widespread calcinosis cutis.

Trisomy 18 is the second most common autosomal trisomy, associated with high mortality, with only 5-10% of affected individuals surviving beyond the first year of life. Consequently, comorbidities in long-term survivors are rarely reported. We describe the case of a 6-year-old East Asian girl with trisomy 18 who presented with calcinosis cutis. The patient initially developed a firm, non-tender subcutaneous nodule on the left wrist, which later spread to the forearm and upper arm. Physical examination and imaging revealed extensive subcutaneous nodules in other extremities. High-frequency ultrasonography showed hypoechogenic masses with posterior acoustic shadows, while skin biopsy revealed fat necrosis and calcium deposition. No significant abnormalities were detected in the levels of calcium, phosphorus, parathyroid hormone or vitamin D. Based on laboratory findings and the patient's medical history, metabolic, nutritional, inflammatory and iatrogenic causes of calcinosis cutis were unlikely. Consequently, the condition was classified as either dystrophic or idiopathic calcinosis cutis. The patient was managed as an outpatient without specific treatment. This report discusses the mechanisms of calcinosis cutis and the reasons for the limited research on its association with trisomy 18. To our knowledge, this is the first report describing calcinosis cutis as a concomitant condition in a paediatric patient with trisomy 18, and it is anticipated that increased awareness of this disease may lead to a rise in reported cases in the future.

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CiteScore
1.70
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