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題 名 | Paraneoplastic Pemphigus=伴腫瘤性天疱瘡 |
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作 者 | 陳柏宏; 陳國熏; 許晴哲; 孫玉蘭; 余幸司; | 書刊名 | 中華皮膚科醫學雜誌 |
卷 期 | 18:1 2000.03[民89.03] |
頁 次 | 頁47-53 |
分類號 | 415.68 |
關鍵詞 | 伴腫瘤性天疱瘡; 自體免疫性水疱症; Paraneoplastic pemphigus; Squamous cell carcinoma; Immunofluorescence; |
語 文 | 英文(English) |
中文摘要 | 伴腫瘤性天疱瘡是一非常少見和腫瘤有關的自體免疫性水疱症。在此我們報告一病例,其臨床表現為口腔黏膜潰瘍及四肢與軀幹上有多發性表淺性潰瘍合併邊緣浮起之紅斑。初步診斷為Stevens-Johnson症候群。然而在組織病理上則有基底膜上裂隙以及裂隙內有棘層鬆解細胞存在。直接免疫螢光檢查發現免疫球蛋白IgG及補體C3沉積於表皮內細胞間空隙與基底膜,而間接免疫螢光則是在老鼠膀胱及大腸上皮細胞間隙有螢光反應。根據以上種種特徵,我們懷疑此患者為一例伴腫瘤性天疱癐。為此我們安排上消化道鋇劑攝影及消化道內視鏡檢查,發現在食道中段有一長達7公分的息肉狀腫瘤,病理證實其為食道鱗狀上皮癌。其口腔皮膚病灶在高劑量Prednisolone(3.5 mg/kg/day)與腫瘤放射線治療下獲得改善,因此我們逐步減低類固醇劑量至5mg/day終至停藥,但不幸的其黏膜皮膚病灶又再度發生,於是只好再增加Prednisolone劑量(1.5mg/kg/day)以控制其皮疹。然而經由氣管鏡檢查發現腫瘤已經轉移至左支氣管,患者因食道腫瘤破裂引發大量出血因而死已。 |
英文摘要 | Paraneoplastic pemphigus is a rare neoplasia-associated autoimmune bullous disease. Here we report a case of this unusual disorder with erosions over oral mucosa, and multiple erosive erythematous plaques with elevated margins over the extremities and trunk. Initially, Stevens-Johnson syndrome was impressed. However, the histopathology showed a supra-basal clef and a lot of acantholytic keratinocytes within the cleft. Direct immunofluorescence revealed depositions of IgG and C3 in the intercellular spaces and basement membrane zone. Indirect immunofluorescence showed positive intercellular deposition on the mucosal layer of the rat urinary bladder and rat colon. Paraneoplastic pemphigus was highly suspected according to the characteristic features mentioned above. We arranged upper GI series and panendoscopy. A polypoid tumor mass about 7 cm in length was demonstrated in the middle third portion of the esophagus. Pathological study revealed squamous cell carcinoma of esophagus. The mucocutaneous lesions improved gradually under the treatments of high dose prednisolone (3.5 mg/kg/day) and radiotherapy for esophageal tumor. Therefore, we tapered the high dose steroid to a very low dose (5 mg/day). Unfortunately, the mucocutaneous lesions recurred when we stopped the low dose steroid. Thus the dose of prednisolone was increased to 1.5 mg/kg/day to control the lesions. However, the bronchoscopy showed metastasis of the tumor to the left main bronchus. Later, he suddenly died of rupture of esophageal tumor and massive bleeding. |
本系統中英文摘要資訊取自各篇刊載內容。