von Dücker Laura, Fleischer Mariella, Stutz Nathalie, Thieme Markus, Witte Mareike, Zillikens Detlef, Sadik Christian D, Terheyden Patrick
Department of Dermatology, Allergy, and Venereology, University of Lübeck, Lübeck, Germany.
Front Oncol. 2020 Feb 19;10:133. doi: 10.3389/fonc.2020.00133. eCollection 2020.
Primary Cutaneous Gamma-Delta (γδ) T-Cell Lymphoma (PCGDTCL) is a rare primary cutaneous lymphoma of aggressive nature. Only a few cases with an initially indolent course over years have been published. PCGDTCL can mimic diseases with benign behavior in their clinical and histopathological presentation, such as lupus erythematosus profundus, but also other lymphomas, for example subcutaneous panniculitis-like T-cell lymphoma. In our patient, the results of histopathological, immunofluorescence microscopy, and clinical examinations of early lesions first led to the diagnosis of lupus erythematosus profundus. Two years after this diagnosis and 6 years after the first clinical symptoms appeared, the disease progressed with erosive and ulcerating plaques and a PCGDTCL with hemophagocytic syndrome with an aggressive course was diagnosed. A distinct correlation of clinical, histopathological, immunohistochemical, and molecular-pathological examinations is needed to differentiate between the potentially malignant and benign diseases. Re-biopsies of different skin lesions in uncertain cases are strongly indicated. This case demonstrates that an indolent clinical phenotype can precede an aggressive clinical course in PCGDTCL.
原发性皮肤γ-δ（γδ）T细胞淋巴瘤（PCGDTCL）是一种罕见的侵袭性原发性皮肤淋巴瘤。仅有少数病例报道显示其病程最初数年呈惰性。PCGDTCL在临床和组织病理学表现上可类似具有良性行为的疾病，如深部红斑狼疮，但也可类似其他淋巴瘤，例如皮下脂膜炎样T细胞淋巴瘤。在我们的患者中，早期病变的组织病理学、免疫荧光显微镜检查及临床检查结果最初诊断为深部红斑狼疮。在该诊断后两年且首次出现临床症状6年后，病情进展为糜烂性和溃疡性斑块，诊断为伴噬血细胞综合征的侵袭性病程的PCGDTCL。需要临床、组织病理学、免疫组织化学及分子病理学检查之间有明确的相关性，以区分潜在的恶性和良性疾病。对于不确定的病例，强烈建议对不同皮肤病变进行再次活检。该病例表明，PCGDTCL中惰性临床表型可先于侵袭性临床病程出现。
