Distinct capillaroscopic findings in a patient with systemic sclerosis: Possible association with a benign disease course

IF 1 Q4 RHEUMATOLOGY Egyptian Rheumatologist Pub Date : 2025-06-01 Epub Date: 2025-04-12 DOI:10.1016/j.ejr.2025.04.002
Angelo Nigro , Dell’Edera Domenico , Nicoletti Giuseppe
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Abstract

Background

Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities and fibrosis. Capillaroscopy is a key tool for assessing microvascular changes in SSc, often revealing characteristic patterns with prognostic significance. While the presence of a “scleroderma pattern” is well-documented, its localization to a single digit without systemic involvement is an unusual finding.

Aim of the work

This report highlights a unique capillaroscopic presentation in a patient with anti-centromere antibody-positive SSc, emphasizing its potential prognostic significance.

Case report

A 66-year-old Caucasian female was diagnosed with anti-centromere antibody-positive SSc six years ago. The diagnosis was supported by ANA positivity (1:1280, centromeric pattern), Raynaud’s phenomenon, and transient hand edema. Capillaroscopic examination revealed a “scleroderma pattern” with prominent megacapillaries exclusively on the second finger of the right hand, with no microhemorrhages. The remaining fingers showed a non-specific capillaroscopic pattern. Clinically, the patient has a benign disease course, with no skin sclerosis, digital ulcers, or systemic involvement.

Conclusion

This case highlights the relevance of localized capillaroscopic abnormalities in SSc. The absence of systemic involvement despite the capillaroscopic findings raises questions about their prognostic significance. Further research is needed to clarify their implications.
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系统性硬化症患者的独特毛细血管镜检查结果:可能与良性病程有关
背景:系统性硬化症(SSc)是一种以血管异常和纤维化为特征的自身免疫性疾病。毛细管镜检查是评估SSc微血管变化的关键工具,通常可以揭示具有预后意义的特征性模式。虽然“硬皮病模式”的存在是有案可查的,但其定位于单指而不累及全身是一种不寻常的发现。本报告强调了抗着丝粒抗体阳性SSc患者的独特毛细血管镜表现,强调了其潜在的预后意义。病例报告一名66岁的白人女性在6年前被诊断为抗着丝粒抗体阳性的SSc。ANA阳性(1:1280,着丝点型)、雷诺现象和短暂性手部水肿支持诊断。毛细血管镜检查显示“硬皮病型”,仅在右手食指有明显的大毛细血管,未见微出血。其余手指显示非特异性毛细血管镜模式。临床表现为良性病程,无皮肤硬化、指部溃疡或全身受累。结论本病例强调了SSc的局限性毛细血管镜异常的相关性。尽管有毛细血管镜检查结果,但没有全身性累及,这引起了对其预后意义的质疑。需要进一步的研究来澄清它们的含义。
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来源期刊
Egyptian Rheumatologist
Egyptian Rheumatologist RHEUMATOLOGY-
CiteScore
2.00
自引率
22.20%
发文量
77
审稿时长
39 weeks
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