Obara Koya, Mii Sumiyuki, Amoh Yasuyuki
Department of Dermatology, Kitasato University School of Medicine, Kanagawa, Japan.
J Cutan Pathol. 2019 Feb;46(2):148-151. doi: 10.1111/cup.13386. Epub 2018 Dec 10.
Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS), is a rare, aggressive, fatal type of cutaneous T-cell lymphoma. The clinical presentation of pcPTCL-NOS is characterized by generalized plaques, nodules or tumors but ulcers are uncommon. We report an atypical case of pcPTCL-NOS with cytotoxic protein expression, presenting as multiple ulcers on the entire body. A 48-year-old man first presented with pruritic papules on the trunk. The papules gradually increased in number and became ulcerated. We finally diagnosed pcPTCL-NOS because of diffuse dermal infiltration of medium- to large-sized pleomorphic CD4 positive lymphoid cells. Ulceration suggests infiltration of lymphoid cells expressing cytotoxic proteins, which can induce apoptosis in the epidermis and dermis. Our patient died of bacterial sepsis that invaded from the uncontrollable ulcers. A suspicion of pcPTCL-NOS is needed when encountering clinical pictures of refractory multiple ulcers and a biopsy should always be performed, because treatment delay may lead to a very poor prognosis.
原发性皮肤外周T细胞淋巴瘤，非特殊类型（pcPTCL-NOS），是一种罕见、侵袭性、致命的皮肤T细胞淋巴瘤。pcPTCL-NOS的临床表现以泛发性斑块、结节或肿瘤为特征，但溃疡并不常见。我们报告了一例具有细胞毒性蛋白表达的非典型pcPTCL-NOS病例，表现为全身多处溃疡。一名48岁男性最初表现为躯干上的瘙痒性丘疹。丘疹数量逐渐增多并发生溃疡。最终我们诊断为pcPTCL-NOS，原因是真皮中弥漫性浸润了中等至大型多形性CD4阳性淋巴细胞。溃疡提示表达细胞毒性蛋白的淋巴细胞浸润，其可诱导表皮和真皮中的细胞凋亡。我们的患者死于因无法控制的溃疡而侵入的细菌性败血症。遇到难治性多发性溃疡的临床症状时，需要怀疑pcPTCL-NOS，并且应始终进行活检，因为治疗延迟可能导致预后极差。
