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奥菲居氏皲裂性红皮病。

Papuloerythroderma of Ofuji.

机构信息

Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

出版信息

Clin Dermatol. 2021 Mar-Apr;39(2):248-255. doi: 10.1016/j.clindermatol.2020.10.017. Epub 2020 Oct 17.

DOI:10.1016/j.clindermatol.2020.10.017
PMID:34272018
Abstract

Papuloerythroderma of Ofuji (PEO) is a rare skin condition first described in 1984 and characterized by diffuse erythroderma composed of papules coalescing into plaques with sparing of skin folds, known as the deck-chair sign. The disease is almost exclusively seen in the elderly and affects men more frequently than women. Common laboratory findings include peripheral and tissue eosinophilia, elevated levels of immunoglobulin E, and lymphopenia. The diagnosis entails exclusion of potentially causative pathologies, including drug intake, atopy, malignancy, and infection. These factors have frequently been found in association with PEO, but their role in the etiopathogenesis of the disease is poorly understood. A dysregulated immune system, with particular involvement of T-helper (Th)2 and Th22 cells, seems to be important in the development of PEO. Controversy exists as to whether PEO exists as an independent entity or as a clinical pattern of a variety of distinct conditions. Treatment necessitates first addressing any coexisting circumstances that may have a causal relationship with PEO. In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.

摘要

奥菲古氏斑状红皮病(PEO)是一种罕见的皮肤疾病,于 1984 年首次描述,其特征为弥漫性红斑,由融合成斑块的丘疹组成,褶皱处皮肤保留,即“躺椅征”。该病几乎仅见于老年人,且男性发病率高于女性。常见的实验室发现包括外周和组织嗜酸性粒细胞增多、免疫球蛋白 E 水平升高和淋巴细胞减少。诊断需要排除可能的病因,包括药物摄入、特应性、恶性肿瘤和感染。这些因素常与 PEO 相关,但它们在疾病发病机制中的作用尚不清楚。免疫系统失调,特别是辅助性 T 细胞(Th)2 和 Th22 细胞的参与,似乎在 PEO 的发展中很重要。目前尚存在争议,即 PEO 是否作为一种独立实体存在,还是作为各种不同疾病的临床模式存在。治疗需要首先解决任何可能与 PEO 有因果关系的共存情况。在特发性病例中,已使用局部和口服皮质类固醇、紫外线疗法和免疫抑制/免疫调节疗法,但结果不一。需要进一步的研究来深入了解疾病过程,并为诊断评估和治疗制定指南。

相似文献

1
Papuloerythroderma of Ofuji.奥菲居氏皲裂性红皮病。
Clin Dermatol. 2021 Mar-Apr;39(2):248-255. doi: 10.1016/j.clindermatol.2020.10.017. Epub 2020 Oct 17.
2
Rapid and successful effects of combining dupilumab with ultraviolet B radiation therapy in the treatment of Papuloerythroderma of Ofuji.奥夫京氏红皮病性银屑病采用度普利尤单抗联合中波紫外线治疗的快速、显著疗效。
Australas J Dermatol. 2023 Aug;64(3):e241-e244. doi: 10.1111/ajd.14078. Epub 2023 May 27.
3
[Ofuji papuloerythroderma: PUVA bath treatment].奥富士丘疹性红皮病:补骨脂素紫外线A光浴治疗
Hautarzt. 1999 May;50(5):360-2. doi: 10.1007/s001050050921.
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[Papuloerythroderma of Ofuji with urticarial lesions].伴有荨麻疹样损害的Ofuji丘疹性红皮病
J Dtsch Dermatol Ges. 2004 Jun;2(6):443-7.
5
Treatment of papuloerythroderma of Ofuji with Re-PUVA: a case report and review of the therapy.补骨脂素联合紫外线A再治疗Ofuji丘疹性红皮病:一例报告及治疗回顾
J Eur Acad Dermatol Venereol. 2004 Jul;18(4):480-3. doi: 10.1111/j.1468-3083.2004.00930.x.
6
Papuloerythroderma of Ofuji in a Young Man.一名年轻男性的Ofuji丘疹性红皮病
Cureus. 2023 Mar 23;15(3):e36598. doi: 10.7759/cureus.36598. eCollection 2023 Mar.
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Papuloerythroderma of Ofuji associated with acute myeloid leukaemia.与急性髓系白血病相关的Ofuji丘疹性红皮病。
Clin Exp Dermatol. 2003 May;28(3):277-9. doi: 10.1046/j.1365-2230.2003.01260.x.
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Ofuji papuloerythroderma: a new European case.大丘疹性红皮病:一例新的欧洲病例。
Dermatology. 1993;186(3):190-2. doi: 10.1159/000247343.
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Ofuji papuloerythroderma: report of a European case.大丘疹性红皮病:1例欧洲病例报告。
Dermatology. 1993;186(3):187-9. doi: 10.1159/000247342.
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Papuloerythroderma of Ofuji in a 41-year-old woman.一名41岁女性的Ofuji丘疹性红皮病。
Postepy Dermatol Alergol. 2013 Oct;30(5):324-8. doi: 10.5114/pdia.2013.38364. Epub 2013 Oct 30.

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JAAD Case Rep. 2024 Nov 29;55:80-82. doi: 10.1016/j.jdcr.2024.11.021. eCollection 2025 Jan.
2
Deciphering the Etiologies of Adult Erythroderma: An Updated Guide to Presentations, Diagnostic Tools, Pathophysiologies, and Treatments.解读成人红皮病的病因:临床表现、诊断工具、病理生理学和治疗方法的最新指南。
Am J Clin Dermatol. 2024 Nov;25(6):927-950. doi: 10.1007/s40257-024-00886-9. Epub 2024 Sep 30.
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A unique case of paraneoplastic lymphomatoid Papuloerythroderma of Ofuji with atypical clinical features.
一例具有非典型临床特征的Ofuji副肿瘤性淋巴样丘疹性红皮病的独特病例。
JAAD Case Rep. 2024 Feb 20;50:102-107. doi: 10.1016/j.jdcr.2024.02.009. eCollection 2024 Aug.
4
Papuloerythroderma of Ofuji in a Young Man.一名年轻男性的Ofuji丘疹性红皮病
Cureus. 2023 Mar 23;15(3):e36598. doi: 10.7759/cureus.36598. eCollection 2023 Mar.