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[慢性良性家族性天疱疮]

[Chronic benign familial pemphigus].

作者信息

Kovalev V M, Tkachenko L A, Krivenko Z F, Stefanovskaia L P

出版信息

Vestn Dermatol Venerol. 1989(8):65-7.

PMID:2816037
Abstract

A female patient aged 50 is described, in whom candidiasis of the large folds has been erroneously diagnosed for 10 years; the diagnosis has not been confirmed by bacteriologic analysis, the therapy has been ineffective, the recurrences developed every year. The patient has developed symmetrical erythematous infiltrative areas in the armpits, groin, and in the folds under mammary glands, with solitary bullae up to 0.5-1.0 cm in diameter, with transparent contents, with thin flaccid covers and fragments thereof, as well as small pigmented vegetation. Basing on the histologic findings (suprabasal vesicles above the basal layer, acantholysis in the epidermis, and negligible lymphocytic infiltrate in the derma), Gougerot-Hailey-Hailey's disease has been diagnosed. The familial nature of the disease could not be established. Therapy with diphenhydramine, methyluracil, dimocifon, aloe extract, and aniline dyes, administered for 20 days, has resulted in clinical cure.

摘要

描述了一位50岁的女性患者,其大褶皱处念珠菌病被误诊长达10年;细菌学分析未证实该诊断,治疗无效,每年都会复发。患者腋窝、腹股沟和乳腺下褶皱处出现对称的红斑浸润区,有直径达0.5 - 1.0厘米的孤立大疱,疱内容物透明,疱盖薄且松弛及其碎片,还有小的色素沉着性赘生物。根据组织学检查结果(基底层上方的基底上水疱、表皮棘层松解以及真皮中淋巴细胞浸润可忽略不计),诊断为 Gougerot - Hailey - Hailey病。该病的家族性无法确定。使用苯海拉明、甲基尿嘧啶、地莫昔芬、芦荟提取物和苯胺染料进行了20天的治疗,实现了临床治愈。

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