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伴有荨麻疹样损害的Ofuji丘疹性红皮病

[Papuloerythroderma of Ofuji with urticarial lesions].

作者信息

Pullmann Susanne, Luger Thomas, Metze Dieter

机构信息

Klinik und Poliklinik für Hautkrankheiten des Universitätsklinikums Münster.

出版信息

J Dtsch Dermatol Ges. 2004 Jun;2(6):443-7.

Abstract

Papuloerythroderma of Ofuji is a rare disease that mainly affects older men of Asiatic origin. Clinically, it is marked by highly pruritic papules which form larger plaques or may give rise to erythroderma. Larger body folds of the abdomen are typically spared (deckchair sign). There is a dispute whether papuloerythroderma of Ofuji represents an own entity or should be regarded as a variant of other inflammatory, neoplastic, or paraneoplastic dermatoses. We present a case of papuloerythroderma of Ofuji in a European patient in which the clinical course as well as the laboratory and histology suggested a relation to mycosis fungoides. Our patient developed pruritic urticarial plaques when systemic corticosteroids were tapered, but cleared complete with cyclosporin A.

摘要

尾状丘疹性红皮病是一种罕见疾病,主要影响亚洲裔老年男性。临床上,其特征为高度瘙痒的丘疹,这些丘疹可形成更大的斑块或引发红皮病。腹部较大的身体褶皱处通常不累及(躺椅征)。尾状丘疹性红皮病是一种独立疾病还是应被视为其他炎症性、肿瘤性或副肿瘤性皮肤病的一种变体,目前存在争议。我们报告了一例欧洲患者的尾状丘疹性红皮病病例,该病例的临床病程以及实验室和组织学检查提示与蕈样肉芽肿有关。我们的患者在全身糖皮质激素逐渐减量时出现瘙痒性荨麻疹斑块,但使用环孢素A后完全消退。

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