鳞状细胞癌并发隐性营养不良性大疱性表皮松解症-哈洛佩奥-西门子型：4例报告
Squamous cell carcinoma complicating recessive dystrophic epidermolysis bullosa-Hallopeau-Siemens: a report of four cases.
作者信息
Dammak Anis, Zribi Jihen, Boudaya Sonia, Mseddi Madiha, Meziou Taha Jalel, Masmoudi Abderrahmen, Ellouze Zoubair, Keskes Hassib, Turki Hamida
机构信息
Dermatology Department, Hedi Chaker Hospital, Sfax, Tunisia.
出版信息
Int J Dermatol. 2009 Jun;48(6):588-91. doi: 10.1111/j.1365-4632.2009.04006.x.
BACKGROUND
Recessive dystrophic epidermolysis bullosa (RDEB), an autosomal-recessive genodermatosis, is one of the more severe forms of the epidermolysis bullosa dystrophica group, and is characterized by generalized blistering of the skin and mucous membranes. Cutaneous squamous cell carcinoma is one of the most serious complications of this disease.
METHODS
We report four patients (three females and one male), two of whom were under 20 years of age, suffering from RDEB-Hallopeau-Siemens.
RESULTS
All patients developed well-differentiated squamous cell carcinoma. No metastases were detected. All cases were treated surgically. Fatal evolution was noted in one patient. A second tumor was detected in another patient during the follow-up period. No further tumors or metastases were observed in the other patients.
CONCLUSIONS
Regular clinical and histologic examination of any nodular lesions or non healing ulcers of all patients suffering from RDEB-Hallopeau-Siemens to detect an early malignancy is recommended.
背景
隐性营养不良型大疱性表皮松解症（RDEB）是一种常染色体隐性遗传性皮肤病，是营养不良型大疱性表皮松解症中较严重的类型之一，其特征为皮肤和黏膜广泛水疱形成。皮肤鳞状细胞癌是该疾病最严重的并发症之一。
方法
我们报告了4例患者（3名女性和1名男性），其中2例年龄在20岁以下，均患有Hallopeau-Siemens型RDEB。
结果
所有患者均发生了高分化鳞状细胞癌，未检测到转移。所有病例均接受了手术治疗。1例患者病情进展至死亡。随访期间，另1例患者发现了第二个肿瘤。其他患者未观察到进一步的肿瘤或转移。
结论
建议对所有患有Hallopeau-Siemens型RDEB的患者的任何结节性病变或未愈合溃疡进行定期临床和组织学检查，以早期发现恶性肿瘤。
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