Medenica Ljiljana, Gajić-Veljić Mirjana, Skiljević Dusan, Pesko Predrag
Clinical Center of Serbia, Institute of Dermatology and Venerology, Belgrade, Serbia.
Vojnosanit Pregl. 2008 Jun;65(6):485-7. doi: 10.2298/vsp0806485m.
Acrokeratosis paraneoplastica Bazex (APB) is a very rare disease in the group of obligate paraneoplastic dermatoses, associated mostly with squamous cell carcinoma of the upper aerodigestive tract and metastatic cervical lymphadenopathy. The disease is characterized by violaceous erythemosquamous changes on the acral regions. This entity was first reported by Bazex in 1965. About 160 cases have been presented so far.
We presented a patient with a three-month history of violaceous erythema, edema, erosions and scaling on the acral regions, elbows and knees and severe nail dystrophy. When the diagnosis was established, he did not have any symptom of internal malignancy. Esophagogastroscopy revealed ulcerovegetant lesion of the esophagus, while histology showed squamocellular invasive carcinoma. Surgical tumor removal resulted in significant improvement of skin changes in 15 days. Unfortunately, four months later, extensive skin lesions pointed to metastasis of squamous cell carcinoma.
Skin changes can precede a few years the first manifestations of neoplasia. The course of the disease in our patient proved that APB is a specific marker of underlying malignancy.
巴泽克斯副肿瘤性肢端角化病(APB)是一组严格意义上的副肿瘤性皮肤病中非常罕见的疾病,主要与上消化道鳞状细胞癌和转移性颈部淋巴结病相关。该疾病的特征是肢端部位出现紫红色红斑鳞屑性改变。这一实体于1965年由巴泽克斯首次报道。迄今为止,已报道约160例病例。
我们报告了一名患者,其肢端、肘部和膝部有三个月的紫红色红斑、水肿、糜烂和鳞屑病史,并有严重的甲营养不良。确诊时,他没有任何内部恶性肿瘤的症状。食管胃镜检查发现食管有溃疡型病变,而组织学检查显示为鳞状细胞浸润癌。手术切除肿瘤后15天皮肤病变有明显改善。不幸的是,四个月后,广泛的皮肤病变提示鳞状细胞癌转移。
皮肤改变可在肿瘤的首发表现出现前数年出现。我们患者的病程证明APB是潜在恶性肿瘤的一个特异性标志物。