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斑片状蕈样肉芽肿:1例临床及组织病理学分析并文献复习

Poikilodermatous mycosis fungoides: clinical and histopathological analysis of a case and literature review.

作者信息

Pankratov Oleg, Gradova Svetlana, Tarasevich Svetlana, Pankratov Valentin

机构信息

Department of Dermatology, Belarusian Medical Academy of Post-Graduate Education, Minsk, Belarus.

Department of Dermatology, Belarusian Medical Academy of Post-Graduate Education, Minsk, Belarus. Corresponding author:

出版信息

Acta Dermatovenerol Alp Pannonica Adriat. 2015;24(2):37-41. doi: 10.15570/actaapa.2015.10.

Abstract

Poikilodermatous mycosis fungoides is a rare distinct clinical variant of cutaneous T-cell lymphoma (CTCL), formerly referred to as poikiloderma vasculare atrophicans or parapsoriasis variegata. Mycosis fungoides (MF) is a malignant neoplasm of T-lymphocyte origin, most commonly memory CD4+ T-cells.We report here a patient with generalized poikilodermatous skin lesions whose diagnosis of mycosis fungoides was made only a few years after the onset of his disease due to its bizarre clinical behavior and a natural reluctance to diagnose this disease in children and adolescents.The variability of atypical clinical presentations of MF and its similarity to benign inflammatory and noninflammatory skin disorders may become a source of considerable confusion and controversy, challenging a dermatologist to make a precise diagnosis. Therefore, scrupulous clinicopathological correlation is an absolute necessity.

摘要

斑驳样蕈样肉芽肿是皮肤T细胞淋巴瘤(CTCL)一种罕见的独特临床变体,以前称为萎缩性血管性斑驳病或杂色副银屑病。蕈样肉芽肿(MF)是一种起源于T淋巴细胞的恶性肿瘤,最常见的是记忆性CD4 + T细胞。我们在此报告一名患有全身性斑驳样皮肤病变的患者,由于其怪异的临床行为以及在儿童和青少年中自然存在的不愿诊断该病的情况,其蕈样肉芽肿的诊断在疾病发作后几年才得以确定。MF非典型临床表现的变异性及其与良性炎症性和非炎症性皮肤疾病的相似性可能会造成相当大的混淆和争议,这对皮肤科医生做出准确诊断构成挑战。因此,严谨的临床病理相关性分析是绝对必要的。

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