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在临床诊断为欧富士丘疹性红皮病的患者中排除皮肤T细胞淋巴瘤的重要性:病例系列

The Importance of Excluding Cutaneous T-Cell Lymphomas in Patients with a Working Diagnosis of Papuloerythroderma of Ofuji: A Case Series.

作者信息

Maher Anthony M, Ward Chloé E, Glassman Steven, Litvinov Ivan V

机构信息

Faculty of Medicine, Memorial University, St. John's, Newfoundland, Canada.

Division of Dermatology, University of Ottawa, Ottawa, Ontario, Canada.

出版信息

Case Rep Dermatol. 2018 Feb 21;10(1):46-54. doi: 10.1159/000487473. eCollection 2018 Jan-Apr.

Abstract

Papuloerythroderma of Ofuji (PEO) is an erythroderma-like eruption with flat-topped papules that spare the skin folds (a "deck-chair sign" finding). Many infections, medications, and systemic diseases have been associated with PEO, including cutaneous T-cell lymphomas (CTCL). The relationship between the clinical presentation of PEO and CTCL remains poorly elucidated. Clinical, laboratory, and histopathological data were obtained from the Lymphoma Clinic at the Ottawa Hospital, Canada. We report 5 patients with deck-chair-sign-positive CTCL, mycosis fungoides, and Sézary syndrome variants. We contend that PEO should be viewed as a diagnosis of exclusion and that these patients should be monitored carefully for possible emergence of CTCL. Skin biopsy alone is not sufficient to exclude CTCL in these patients. A skin eruption demonstrating a positive deck-chair sign may signify systemic/leukemic CTCL and, therefore, warrants a thorough investigation, including skin biopsy, flow cytometry, and T-cell receptor clonality studies.

摘要

Ofuji红皮病样丘疹性红皮病(PEO)是一种类似红皮病的皮疹,有平顶丘疹,不累及皮肤褶皱(“躺椅征”表现)。许多感染、药物和全身性疾病都与PEO有关,包括皮肤T细胞淋巴瘤(CTCL)。PEO的临床表现与CTCL之间的关系仍未得到充分阐明。临床、实验室和组织病理学数据来自加拿大渥太华医院淋巴瘤诊所。我们报告了5例具有躺椅征阳性的CTCL、蕈样肉芽肿和Sezary综合征变体患者。我们认为PEO应被视为一种排除性诊断,并且应对这些患者进行密切监测,以防CTCL的可能出现。仅皮肤活检不足以排除这些患者的CTCL。表现为阳性躺椅征的皮肤疹可能意味着系统性/白血病性CTCL,因此,需要进行全面检查,包括皮肤活检、流式细胞术和T细胞受体克隆性研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f49/5869582/c81ce3f48362/cde-0010-0046-g01.jpg

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