Di Prete Monia, Michelerio Andrea, Lora Viviana, Tomasini Carlo Francesco, Cota Carlo
Dermatopathology Laboratory, San Gallicano Dermatological Institute IRCCS, Rome.
Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia.
Dermatol Reports. 2024 May 7;16(Suppl 2):10008. doi: 10.4081/dr.2024.10008.
The most prevalent primary cutaneous T-cell lymphoma, mycosis fungoides (MF), is characterized by the development of plaques and nodules after an erythematous patchy phase that is non-specific. An infiltrate of atypical small- to medium-sized cerebriform lymphocytes in the superficial dermis, with variable epidermotropism, is the histopathological hallmark of the disease. In more advanced stages of the illness, large-cell transformation may be seen. Early diagnosis of MF can be very challenging based only on histopathologic or clinical findings, so it is critical to have a clinical-pathological correlation. Many atypical variants of MF that deviate from the classic Alibert-Bazin presentation of the disease have been described over the past 30 years, sometimes with different prognostic and therapeutic implications. Clinically or histopathologically, they can mimic a wide range of benign inflammatory skin disorders. To make a conclusive diagnosis in these cases, it is recommended to take multiple biopsies from various lesions and to carefully correlate the clinical and pathological findings. We have outlined the various facets of the illness in this review, positioning MF as a "great imitator", with an emphasis on the more recently identified variations, differential diagnosis, and its benign mimics.
最常见的原发性皮肤T细胞淋巴瘤——蕈样肉芽肿(MF),其特征是在非特异性的红斑斑片期之后出现斑块和结节。浅表真皮层出现非典型的中小脑回状淋巴细胞浸润,并伴有不同程度的亲表皮现象,是该病的组织病理学特征。在疾病的更晚期,可能会出现大细胞转化。仅基于组织病理学或临床发现,MF的早期诊断可能极具挑战性,因此临床与病理的相关性至关重要。在过去30年中,已描述了许多偏离经典阿利贝尔 - 巴赞病表现的MF非典型变体,有时具有不同的预后和治疗意义。在临床或组织病理学上,它们可模仿多种良性炎症性皮肤病。为了在这些病例中做出确定性诊断,建议从不同病变处进行多次活检,并仔细关联临床和病理发现。我们在本综述中概述了该病的各个方面,将MF定位为“伟大的模仿者”,重点关注最近发现的变体、鉴别诊断及其良性模仿疾病。