一名婴儿同时患有脓皮病和高安氏动脉炎:诊断难题和治疗注意事项。

Gülcan Özomay Baykal, Betül Sözeri
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引用次数: 0

摘要

背景:高安动脉炎(TA)是一种不常见的慢性炎症和自身免疫性疾病,主要影响大血管,尤其是主动脉及其分支。有记录表明,皮肤表现与高安动脉炎有关。坏疽性脓皮病(PG)是一种慢性嗜中性皮肤病,以破坏性、坏死性和疼痛性溃疡为特征,主要发生在下肢。PG和TA同时存在的情况极为罕见,大多数报道的病例涉及成年患者。有趣的是,与北美和欧洲人群相比,PG 和 TA 的关联似乎在日本更为常见。伴有 PG 的儿童 TA(c-TA)异常罕见,迄今只有 10 例文献报道:本病例是一名 7 个月大的患者,最初被诊断为 PG。尽管接受了积极的免疫抑制治疗,但患者的急性期反应物仍然很高。虽然腹部超声检查正常,但由于腹部剧痛,患者接受了高级造影检查。主动脉及其分支的对比增强计算机断层扫描血管造影显示广泛的血管受累,与 TA 一致:在本报告中,我们重点介绍了一例随后被诊断为小儿 TA 的小儿 PG。认识到 PG 和 TA 之间的罕见关联非常重要。对患有 PG 的婴儿进行彻底评估并及时诊断为 TA,可指导进一步检查并预防血管并发症。
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Concurrent pyoderma gangrenosum and Takayasu arteritis in an infant: diagnostic challenges and treatment considerations.

Background: Takayasu arteritis (TA) is an uncommon chronic inflammatory and autoimmune disease primarily affecting large vessels, particularly the aorta and its branches. Skin manifestations have been documented in association with TA. Pyoderma gangrenosum (PG) is a chronic neutrophilic dermatosis characterized by destructive, necrotizing, and painful ulcers, predominantly found on the lower extremities. The coexistence of PG and TA is extremely rare, with most reported cases involving adult patients. Interestingly, the association between PG and TA appears to be more common in Japan compared to North American and European populations. Childhood TA (c-TA) accompanied by PG is exceptionally rare, with only 10 cases reported in the literature thus far.

Case report: We present the case of a 7-month-old patient initially diagnosed with PG. Despite aggressive immunosuppressive therapy, the patient`s high acute phase reactants remained elevated. Although the abdominal ultrasound was normal, advanced imaging was performed due to severe abdominal pain. Contrastenhanced computerized tomography angiography of the aorta and its branches revealed extensive vascular involvement consistent with TA.

Conclusion: In this report, we highlight an infantile case of PG that was subsequently diagnosed as infantile TA. Recognizing the rare association between PG and TA is important. Thorough evaluation and prompt diagnosis of TA in infants with PG can guide further investigations and prevent vascular complications.

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