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红皮病型蕈样肉芽肿合并高嗜酸性粒细胞综合征:一种罕见的表现。

Erythrodermic mycosis fungoides with hypereosinophilic syndrome: a rare presentation.

作者信息

Chaudhary Savita, Bansal Cherry, Ranga Upasna, Singh Kk

机构信息

Department of Dermatology, Era's Lucknow Medical College and Hospital, Lucknow 226003, Uttar Pradesh, India.

出版信息

Ecancermedicalscience. 2013 Aug 5;7:337. doi: 10.3332/ecancer.2013.337. Print 2013.

DOI:10.3332/ecancer.2013.337
PMID:23940492
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3738031/
Abstract

Mycosis fungoides (MF) is the most common variant of primary cutaneous T-cell lymphoma (CTCL). It is generally associated with an indolent clinical course and characterised by well-defined clinicopathological features. Although rare, CTCLs constitute 65% of all cutaneous lymphoid malignancies, of which 50% are patients with MF. The erythrodermic variants of MF, a malignancy of mature, skin homing and clonal T lymphocytes, usually present in mid to late adulthood. Association with hypereosinophilia is important in prognosis. We report a case of erythrodermic MF with hypereosinophilic syndrome in a 22-year-old female presenting with gradually progressive intractable erythroderma with intensely pruritic multiple papules, plaques, and nodules involving more than 90% of body surface area. Diagnosis was confirmed by histopathological examination and immunophenotyping from multiple skin biopsies.

摘要

蕈样肉芽肿(MF)是原发性皮肤T细胞淋巴瘤(CTCL)最常见的变异型。它通常与惰性临床病程相关,具有明确的临床病理特征。虽然罕见,但CTCL占所有皮肤淋巴恶性肿瘤的65%,其中50%是MF患者。MF的红皮病型变异型是成熟的、归巢于皮肤的克隆性T淋巴细胞的恶性肿瘤,通常出现在成年中期至晚期。与嗜酸性粒细胞增多症相关对预后很重要。我们报告一例22岁女性红皮病型MF合并嗜酸性粒细胞增多综合征,患者表现为逐渐进展的顽固性红皮病,伴有剧烈瘙痒的多个丘疹、斑块和结节,累及身体表面积超过90%。通过多次皮肤活检的组织病理学检查和免疫表型分析确诊。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/2408573da100/can-7-337fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/b58dba082d71/can-7-337fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/78eb6c7d2b33/can-7-337fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/2408573da100/can-7-337fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/b58dba082d71/can-7-337fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/78eb6c7d2b33/can-7-337fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee4e/3738031/2408573da100/can-7-337fig3.jpg

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本文引用的文献

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Refining the definition of hypereosinophilic syndrome.细化嗜酸性粒细胞增多综合征的定义。
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Mycosis fungoides: a dermatological masquerader.
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Br J Dermatol. 2007 Jan;156(1):1-10. doi: 10.1111/j.1365-2133.2006.07526.x.
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Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas.红皮病型皮肤T细胞淋巴瘤的最新进展:皮肤淋巴瘤国际协会报告
J Am Acad Dermatol. 2002 Jan;46(1):95-106. doi: 10.1067/mjd.2002.118538.
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Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality.蕈样肉芽肿报告发病率及相关死亡率的20年趋势。
Am J Public Health. 1999 Aug;89(8):1240-4. doi: 10.2105/ajph.89.8.1240.
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EORTC classification for primary cutaneous lymphomas: a proposal from the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer.欧洲癌症研究与治疗组织原发性皮肤淋巴瘤分类:欧洲癌症研究与治疗组织皮肤淋巴瘤研究组的一项提议。
Blood. 1997 Jul 1;90(1):354-71.
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Pre-Sézary erythroderma evolving to Sézary syndrome. A report of seven cases.前驱赛塞里红皮病演变为赛塞里综合征。7例报告。
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Mycosis fungoides in the United States. Increasing incidence and descriptive epidemiology.美国蕈样肉芽肿。发病率上升及描述性流行病学
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