口內大疱型全身性紅斑性狼瘡－病例報告

Intraoral Bullous Systemic Lupus Erythematosus-A Case Report

張婷菡(Ting-Han Chang) ； 李曉屏(Shiao-Pieng Lee) ； 張燕清(Yen-Ching Chang) ； 夏毅然(Yi-Jan Hsia) ； 侯宗昀(Zong-Yun Hou) ； 葉松峰(Song-Feng Ye) ； 高鴻偉(Hong-Wei Gao) ； 陳元武(Yuan-Wu Chen)

《臺灣口腔顎面外科學會雜誌》 21卷1期 (2010/03) Pp. 35-44

https://doi.org/10.7104/TJOMS.201003.0035

摘要

本病例報告爲一大疱型全身性紅斑性狼瘡之案例。患者因在口內突然發現巨大內含出血性液體之大疱而來求診，追溯其病史，病忠在兩個月前頸部和軀幹就有廣泛且繃緊的水疱形成。直接免疫螢光染色顯示大量C3補體沿著基底膜層沉澱。血液檢查顯示其抗核抗體(ANA)、抗雙股去氧核苷酸(antids DNA)及其它與全身紅斑性狼瘡相關之抗體呈顯著陽性反應，最終診斷爲大疱型全身性紅斑性狼瘡。經高劑量類固醇治療三天後，口內及全身症狀明顯改善。大疱型全身性紅斑性狼瘡的表徵在全身性紅斑性狼瘡患者身上非常罕見，本篇報告回顧了近期文獻，並討論自體免疫疾病之鑑別診斷。

關鍵字

大疱型全身性紅斑性狼瘡 ； 小水疱 ； 天疱瘡

並列摘要

We report a case of bullous systemic lupus erythematosus. The patient suddenly occurred with a huge bulla containing hemorrhagic fluid at the buccal mucosa and widespread, tense and fluid-filling blistering eruption on the trunk and neck. The skin biopsy showed subepidermal blister with a prominent neutrophilic infiltration. Direct immunofluorescence revealed C3 deposition along the basement membrane zone. Investigations revealed positive antinuclear antibodies, anti-ds DNA, anti-Ro, La, RNP and Sm. A diagnosis of bullous systemic lupus erythematosus was made. Rapid resolution of the blisters occurred following treatment with high dose of steroid 45 mg daily. Bullous systemic lupus erythematosus is a rare manifestation of systemic lupus erythematosus. We reviewed the recent literatures and discussed the condition of the distinctive features of this condition and differentiated them with other blistering disorder of autoimmune diseases.

並列關鍵字

bullous systemic lupus erythematosus ； vesicles ； pemphigus