Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra

被引：162

作者：

Brenner, M. ^{[1
]}

Ruzicka, T. ^{[1
]}

Plewig, G. ^{[1
]}

Thomas, P. ^{[1
]}

Herzer, P.

机构：

[1] Univ Munich, Dept Dermatol, D-80337 Munich, Germany

来源：

BRITISH JOURNAL OF DERMATOLOGY | 2009年 / 161卷 / 05期

关键词：

anakinra; interleukin-1 receptor antagonist; pyoderma gangrenosum; PAPA (pyogenic arthritis; pyoderma gangrenosum and acne) syndrome; AUTOINFLAMMATORY DISORDER; RHEUMATOID-ARTHRITIS; INFLIXIMAB; MUTATIONS; INFLAMMASOME; IMPROVEMENT; ADALIMUMAB; DISEASES; PATHWAY; FEVER;

D O I：

10.1111/j.1365-2133.2009.09404.x

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

The triad of sterile pyogenic arthritis, pyoderma gangrenosum and acne is known by the acronym of PAPA syndrome. It is a rare autosomal dominant disease of early onset. The treatment of pyoderma gangrenosum is challenging as there is often only partial response to systemic glucocorticosteroids and immunosuppressive therapies. We report the rapid and lasting response of pyoderma gangrenosum to the targeted treatment with the recombinant human interleukin-1 receptor antagonist (rHuIL-1Ra) anakinra in a patient with PAPA syndrome.

引用

页码：1199 / 1201

页数：3

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