隐性营养不良型大疱性表皮松解症患者鳞状细胞癌的管理。
Management of squamous cell carcinoma in a patient with recessive-type epidermolysis bullosa dystrophica.
作者信息
Yamada Mizuki, Hatta Naohito, Sogo Kana, Komura Kazuhiro, Hamaguchi Yasuhito, Takehara Kazuhiko
机构信息
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
出版信息
Dermatol Surg. 2004 Nov;30(11):1424-9. doi: 10.1111/j.1524-4725.2004.30440.x.
BACKGROUND
Surgical treatment of a recessive dystrophic epidermolysis bullosa patient is very difficult for both the surgeon and the anesthetist because of the fragility of the skin and abnormal nature of the tumor bed.
OBJECTIVE
We report a case of 54-year-old Japanese recessive dystrophic epidermolysis bullosa patient with squamous cell carcinoma (SCC) of the lateral malleolus.
METHODS
A tumor measuring 5.0 x 5.5 cm was surgically excised. The defect was then reconstructed by full-thickness skin grafting. To avoid airway complications, general anesthesia was administered using a face mask. Because the regional lymph nodes were swollen before surgery, the patient underwent sentinel lymph node biopsy.
RESULTS
The patient remains well with no sign of recurrence or metastasis 7 months after surgery.
CONCLUSION
To preserve activities of daily living, surgery should be performed for squamous cell carcinomas arising in recessive dystrophic epidermolysis bullosa patients.
背景
由于皮肤脆弱以及肿瘤床的异常性质，对于外科医生和麻醉师而言，隐性遗传性营养不良型大疱性表皮松解症患者的手术治疗都极具难度。
目的
我们报告一例54岁日本隐性遗传性营养不良型大疱性表皮松解症患者，其外踝出现鳞状细胞癌（SCC）。
方法
手术切除一个大小为5.0×5.5厘米的肿瘤。然后通过全厚皮片移植修复缺损。为避免气道并发症，使用面罩进行全身麻醉。由于术前区域淋巴结肿大，患者接受了前哨淋巴结活检。
结果
术后7个月，患者情况良好，无复发或转移迹象。
结论
为保留日常生活活动能力，对于隐性遗传性营养不良型大疱性表皮松解症患者发生的鳞状细胞癌应进行手术治疗。
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