淋巴瘤样丘疹病(LyP)伴DUSP22-IRF4重排是一种罕见的,最近描述的以双相生长模式为特征的组织病理学上的LyP变体,具有表皮性的小到中等大小的非典型T细胞和真皮大和转化的T细胞弥漫性表达CD30。具有DUSP22-IRF4重排的LyP可以模仿其他皮肤淋巴增生性疾病,特别是原发性皮肤间变性大细胞淋巴瘤(PCALCL)或转化的真菌病(MF)。与PCALCL或转换后的MF不同,具有DUSP22-IRF4重排的LyP显示惰性临床行为,未经治疗的病变频繁自发消退。因此,重要的是要认识到LyP的这种罕见变体,以避免错误分类,这可能会导致不必要的积极的病人管理。据我们所知,迄今为止,英文文献中仅报道了13例伴有DUSP22-IRF4重排的LyP.在这里,我们描述了一名63岁男性的LyP伴DUSP22-IRF4重排的额外病例,并提供了关于临床的全面文献综述,组织病理学,和这种新实体的分子特征。
Lymphomatoid papulosis (LyP) with DUSP22-IRF4 rearrangement is a rare, recently described variant of LyP histopathologically characterized by a biphasic growth pattern, with epidermotropic small-to-medium-sized atypical T-cells and dermal large and transformed T-cells diffusely expressing CD30. LyP with DUSP22-IRF4 rearrangement can mimic other cutaneous lymphoproliferative disorders, particularly primary cutaneous anaplastic large cell lymphoma (PCALCL) or transformed mycosis fungoides (MF). Unlike PCALCL or transformed MF, LyP with DUSP22-IRF4 rearrangement shows an indolent clinical behavior, with frequent spontaneous regression of untreated lesions. Thus, it is important to recognize this rare variant of LyP to avoid misclassification, which may potentially lead to unnecessarily aggressive patient management. To our knowledge, only 13 cases of LyP with DUSP22-IRF4 rearrangement have been reported to date in the English literature. Herein, we describe an additional case of LyP with DUSP22-IRF4 rearrangement in a 63-year-old man and provide a comprehensive literature
review with regards to the clinical, histopathologic, and molecular features of this novel entity.