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一例青年患者的蕈样肉芽肿病 E 型:一种不常见的疾病。

A case of lymphomatoid papulosis type E in a young adult: An uncommon entity.

机构信息

Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts, USA.

Section of Dermatopathology, Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts, USA.

出版信息

J Cutan Pathol. 2021 May;48(5):694-700. doi: 10.1111/cup.13974. Epub 2021 Feb 18.

DOI:10.1111/cup.13974
PMID:33533041
Abstract

Lymphomatoid papulosis (LyP) type E is a rare variant of the primary cutaneous CD30+ lymphoproliferative disorders, characterized clinically by large necrotic eschar-like lesions and histopathologically by angiodestructive and angioinvasive infiltrates of CD30+ lymphocytes. As in other forms of lymphomatoid papulosis, type E lesions may undergo spontaneous regression after weeks, with frequent recurrences. We report a 21-year old male with an angiodestructive infiltrate of CD30+ lymphocytes manifesting as a papular eruption rather than ulceration, and suggest that this clinical phenotype might be related to the presence of CD4+ lymphocytes in the inflammatory cell infiltrate.

摘要

蕈样肉芽肿病(LyP)E 型是一种罕见的原发性皮肤 CD30+淋巴增生性疾病的变异型,临床上表现为大的坏死性象皮样皮损,组织病理学上表现为 CD30+淋巴细胞的血管破坏性和血管侵袭性浸润。与其他蕈样肉芽肿病类型一样,E 型病变可在数周后自发消退,但常有复发。我们报告了一例 21 岁男性,其 CD30+淋巴细胞的血管破坏性浸润表现为丘疹性发疹而不是溃疡,我们推测这种临床表型可能与炎症细胞浸润中存在 CD4+淋巴细胞有关。

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Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity.伴有T细胞受体γ阳性的E型淋巴瘤样丘疹病。
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Etiopathogenesis, Diagnosis, and Treatment Strategies for Lymphomatoid Papulosis with Particular Emphasis on the Role of the Immune System.
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Cells. 2022 Nov 21;11(22):3697. doi: 10.3390/cells11223697.