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全身型膿疱性乾癬:19個病例之回顧

Generalized Pustular Psoriasis: A Review of 19 Cases

摘要


膿疱性乾癬是一種較嚴重而罕見的乾癬,表皮內因中性球聚集造成大量無菌性膿疱。本文收集19位全身型膿疱性乾癬患者的資料,其中有5位首次出現乾癬即以膿疱性乾癬的型式發作。在50次發作中,確定產生次發性感染的有3次,膿疱培養呈現陽性結果。45%出現低白蛋白血症(14/31),52%出現低血鈣(14/27);31%曾出現高尿酸血症(10/32),但只有三位確實有痛風性關節炎。其中有兩位患者在膿疱發作時合併肝功能異常,是否屬於中性球膽管炎,目前無法確認。雖然上呼吸道感染、懷孕、壓力、服用不明中藥、長期全身投與類固醇之斷除徵症等情況可能引發膿疱發作,但多數患者(40%)仍無法找出明確誘發因素。在預後方面,有11位病人不曾有過完全緩解的情形,3位在6個月內復發,5位曾經歷6個月以上的緩解期。 (中華皮誌21:317-325,2003)

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並列摘要


Pustular psoriasis is a rare but severe type of psoriasis. Neutrophils accumulate in the epidermis and form massive sterile macroscopic pustules. We herein report 19 cases of generalized pustular psoriasis. The pustular lesions arose de novo in 5 patients. Among 50 episodes of attack, secondary bacterial infection of pustular was highly suspected in 3.Forty-five percent showed hypoalbuminemia (14/31) while 52% disclosed hypocalcemia (14/27). Although 31% of patients revealed hyperuricemia (10/32), only 3 of them developed gouty arthritis. Two patients had liver function impairment. Whether it resulted from neutrophilic cholangitis merits further studies. Upper respiratory tract infection, pregnancy, stress, herb drugs, or withdrawal from prolonged use if systemic steroids may have provoked flares of pustular psoriasis in our patients. However, most of them showed no obvious predisposing factors. Complete remission never occurred in 11 patients. Of those cleared or much improved after therapy, 3 relapsed within 6 months, and 5 maintained remission for more than 6 months. (Dermatol Sinica 21:317-325,2003)

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