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蕈样肉芽肿和肉芽肿性松弛皮肤：具有不同临床表型的单一实体。

Mycosis Fungoides and Granulomatous Slack Skin: A Single Entity With Distinct Clinical Phenotypes.

机构信息

St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, United Kingdom.

Department of Pathology, Southampton General Hospital, Southampton, United Kingdom; and.

出版信息

Am J Dermatopathol. 2024 Jan 1;46(1):14-20. doi: 10.1097/DAD.0000000000002590. Epub 2023 Nov 21.

DOI:10.1097/DAD.0000000000002590
PMID:37987779
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10718212/
Abstract

Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs). 1 These overlapping entities have shared clinical and histopathologic features which can present a diagnostic challenge. The dominance of the granulomatous infiltrate and the often sparse lymphocytic infiltrate frequently with minimal cytological atypia are features that distract from the correct diagnosis, even when raised by the clinician. We describe the clinical and histopathologic characteristics of 3 cases of granulomatous cutaneous T-cell lymphoma, illustrate the close clinical and pathologic relationship between GMF and GSS and emphasize the diagnostic difficulties that the granulomatous infiltrate can present. Furthermore, we demonstrate, for the first time, considerable elastolysis in a significant proportion of classical (Alibert-Bazin) MF lesions and therefore postulate that the differences observed between GMF and GSS are one of degree and secondary to their anatomic location rather than reflecting meaningful separate entities.

摘要

肉芽肿性皮肤 T 细胞淋巴瘤包括伴有显著肉芽肿性炎症（GMF）和肉芽肿性松弛皮肤（GSS）的蕈样肉芽肿，在世界卫生组织分类中被列为蕈样肉芽肿（MFs）的一个亚型。1 这些重叠实体具有共同的临床和组织病理学特征，这可能带来诊断挑战。肉芽肿浸润的主导地位以及通常稀疏的淋巴细胞浸润，常常伴有最小的细胞学异型性，这些特征会干扰正确的诊断，即使临床医生提出了这些特征。我们描述了 3 例肉芽肿性皮肤 T 细胞淋巴瘤的临床和组织病理学特征，说明了 GMF 和 GSS 之间密切的临床和病理关系，并强调了肉芽肿浸润可能带来的诊断困难。此外，我们首次证明在相当一部分经典（Alibert-Bazin）MF 病变中存在大量弹性溶解，因此我们推测 GMF 和 GSS 之间观察到的差异是程度上的差异，是由于它们的解剖位置，而不是反映有意义的独立实体。

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本文引用的文献

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Granulomatous mycosis fungoides, a rare subtype of cutaneous T-cell lymphoma.肉芽肿性蕈样霉菌病，一种罕见的皮肤T细胞淋巴瘤亚型。
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Granulomatous mycosis fungoides presenting as poikiloderma.蕈样肉芽肿表现为异色性皮病。
Clin Exp Dermatol. 2009 Aug;34(6):718-20. doi: 10.1111/j.1365-2230.2008.03013.x. Epub 2008 Dec 2.
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Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC).肉芽肿性蕈样霉菌病和肉芽肿性皮肤松弛症：欧洲癌症研究与治疗组织（EORTC）皮肤淋巴瘤组织病理学特别工作组的多中心研究。
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Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides.皮肤萎缩性蕈样肉芽肿：蕈样肉芽肿的一种新的临床病理变异型。
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