A case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, with cytotoxic phenotype showing multiple ulcers on the entire body
A case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, with cytotoxic phenotype showing multiple ulcers on the entire body
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一例原发性皮肤外周T细胞淋巴瘤，未另说明，具有细胞毒性表型，显示全身多发性溃疡
DOI：
10.1111/cup.13386
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发表时间：
2018
影响因子：
1.7
通讯作者：
Y. Amoh
中科院分区：
文献类型：
--
作者：
K. Obara;Sumiyuki Mii;Y. Amoh
原发性皮肤外周T细胞淋巴瘤，未另行说明（pcPTCL‐NOS），是一种罕见的、侵袭性的、致死性类型的皮肤T细胞淋巴瘤。pcPTCL‐NOS的临床表现以全身斑块、结节或肿瘤为特征，但溃疡不常见。我们报告了一个非典型的pcPTCL-NOS与细胞毒性蛋白表达的情况下，表现为全身多发性溃疡。一名48岁的男性首先出现躯干上的丘疹。丘疹逐渐增多并溃烂。我们最终诊断为pcPTCL‐NOS，因为中等至大型多形性CD 4阳性淋巴细胞弥漫性真皮浸润。溃疡提示表达细胞毒性蛋白的淋巴细胞浸润，可诱导表皮和真皮细胞凋亡。我们的病人死于无法控制的溃疡侵入的细菌性败血症。当遇到难治性多发性溃疡的临床表现时，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非常差。
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.