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NEJM临床图片:副肿瘤性肢端角化症

66岁男性患者,4周前耳朵出现疼痛和水肿,并出现鳞屑。随后手掌和脚底出现瘙痒性角化过度性斑块,病损周围有红肿(见图A,B,C),呈对称性分布。局部涂抹0.05%丙酸氯倍他索乳膏无效。此外,患者左侧颈部有一肿物,近期体重下降4 kg,此外并无显著的全身症状(B症状,如发热、盗汗和体重下降等)。怀疑为Bazex综合征(副肿瘤性肢端角化症),对肿大淋巴结的细胞学活检结果提示低分化鳞状细胞癌。CT/PET-CT提示为原发于口咽部的鳞状细胞癌(肿瘤分期为4a,淋巴结分期2c,无远处转移)。患者接受了为期6周的放化疗,随后皮肤病损得到改善(图D,E,F)。确诊9个月后患者因远处转移相关并发症死亡。



A 66-year-old man presented to the clinic with a 4-week history of painful, edematous, and scaly ears, a condition that was later accompanied by itchy, hyperkeratotic plaques on the palms and soles with surrounding erythema (Panels A, B, and C). The skin lesions were noted to be symmetric. Previous treatment with a 0.05% formulation of clobetasol cream was unsuccessful. He was also discovered to have an enlarged mass on the left side of his neck and weight loss of 4 kg without other B symptoms (i.e., fever, night sweats, and weight loss). Bazex’s syndrome (acrokeratosis paraneoplastica) was suspected, and cytologic analysis of the enlarged lymph node showed metastasis of a poorly differentiated squamous-cell carcinoma. The presence of a squamous-cell carcinoma of the oropharynx (tumor stage 4a, lymph-node stage 2c, and no distant metastasis) was confirmed on computed tomography and positron-emission tomography–computed tomography. The patient underwent chemoradiotherapy for 6 weeks, after which the skin condition cleared (Panels D, E, and F). Nine months after the initial diagnosis, the patient died from complications of metastatic disease.

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