Limited Cutaneous Systemic Sclerosis: Report of One Case

侷限於表皮之全身性硬皮症：病例報告

林明欽(Ming-Ching Lin) ； 傅令嫻(Lin-Shien Fu) ； 黃奎諭(Kuei-Yu Huang) ； 曾崇芳(Ghung-Fang Tsen) ； 劉智民(Jyh-Min Liou) ； 遲景上(Ching-Shiang Chi)

《Acta Paediatrica Taiwanica》 42卷4期 (2001/08) Pp. 248-251

https://doi.org/10.7097/APT.200108.0248

摘要

硬皮症主要是發生在成人的自體免疫疾病，在兒童以局部性硬皮症爲主。我們報告一個少見的兒童限制型全身性硬皮症-發病年齡五歲，臨床症狀是臉，手指，手臂，皮層變硬，皮下鈣化，微血管擴張及雷諾氏現象。實驗室檢查呈現抗核抗體腸性及Sc1-70抗體陽性。目前無內臓器官侵犯現象。但由過去的經驗看來，本病例須長期追蹤。硬皮及雷諾氏現象在Dipyridamole、D-penicillamine、Colchicine及Nifedipine藥物治療下有相當改善。

關鍵字

限制型全身性硬皮症 ； 雷諾氏現象

並列摘要

We report a case of systemic sclerosis, limited type, in a 5 year old boy in Taiwan. He presented with erythema, tight skin over his face and digits, subcutaneous calcification, telangiectasis, as well as Raynauds phenomenon. The laboratory data showed weakly positive antinuclear antibody(ANA), and Topoisomerase 1 antibody (anti-Scl-70) titers. However his esophagogram, CO diffusion capacity, as well as cardiac and renal function were all normal. He suffered from the skin change for 2 to 3 years before visiting our pediatric clinic when he was 7 years old. We gave him Dipyridamole, D-penicillamine, Colchicine, and Nifedipine. There was some remission of the scieroderma and Raynaud’s phenomenon after our treatment.

並列關鍵字

limited cutaneous systemic sclerosis ； Raynaud's phenomenon