Department of Dermatology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
J Am Acad Dermatol. 2012 Oct;67(4):748-59. doi: 10.1016/j.jaad.2011.07.043. Epub 2012 Jan 9.
Primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma is a rare cytotoxic lymphoma characterized clinically by aggressive behavior and histologically by prominent epidermotropism of atypical CD8(+) lymphocytes. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is still awaiting. Herein, we study and analyze the different clinical, histopathological, and immunohistochemical features described in the reported cases. Different therapeutic modalities and their impact on the prognosis of the tumor are also evaluated and presented. We propose two sets of diagnostic criteria. The first comprises constant clinical, histopathological, and immunohistochemical features that are always present in every case, and the combination of which is necessary for the diagnosis. The second set helps to avoid missing cases and includes variable features that may be present in some cases, and to which any emerging finding could be added. Although different therapeutic options have been used, either as single agents or in combinations, there is no standard therapy for primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma and the tumor still represents a therapeutic challenge with very poor prognosis.
原发性皮肤侵袭性 CD8(+) T 细胞淋巴瘤是一种罕见的细胞毒性淋巴瘤，临床上表现为侵袭性行为，组织学上表现为典型的 CD8(+)淋巴细胞显著亲表皮性。尽管不断有新的病例报告，但尚未确立明确的诊断标准，仍在等待最佳治疗方法。在此，我们研究和分析了已报道病例中描述的不同临床、组织病理学和免疫组织化学特征。还评估和介绍了不同的治疗方式及其对肿瘤预后的影响。我们提出了两套诊断标准。第一套标准包括始终存在于每个病例中的恒定临床、组织病理学和免疫组织化学特征，这些特征的组合是诊断所必需的。第二套标准有助于避免漏诊，包括一些病例中可能存在的可变特征，以及可以添加任何新出现的发现。尽管已经使用了不同的治疗方法，无论是单一药物还是联合使用，但对于原发性皮肤侵袭性 CD8(+) T 细胞淋巴瘤还没有标准的治疗方法，该肿瘤仍然是一个具有非常差预后的治疗挑战。
