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表现为致命性大疱性剥脱性红皮病的非地方性落叶型天疱疮。

Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma.

作者信息

Nousari H C, Moresi M, Klapper M, Anhalt G J

机构信息

Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

出版信息

Cutis. 2001 Mar;67(3):251-2.

Abstract

Pemphigus foliaceus is a cutaneous autoimmune blistering disease that is characterized by lower morbidity and mortality than those observed in pemphigus vulgaris or paraneoplastic pemphigus. However, erythrodermic forms of the endemic variant of pemphigus foliaceus have been associated with a higher mortality. We report a case of nonendemic pemphigus foliaceus that presented as fatal bullous exfoliative erythroderma, and thus, we will emphasize the inclusion of this entity in the differential diagnosis and the use of skin direct immunofluorescence in the evaluation of patients with erythroderma.

摘要

落叶型天疱疮是一种皮肤自身免疫性水疱病,其发病率和死亡率低于寻常型天疱疮或副肿瘤性天疱疮。然而,地方性落叶型天疱疮的红皮病型与较高的死亡率相关。我们报告一例非地方性落叶型天疱疮,表现为致命性大疱性剥脱性红皮病,因此,我们将强调在鉴别诊断中纳入该疾病,并在评估红皮病患者时使用皮肤直接免疫荧光检查。

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