Squamous cell carcinoma in junctional and dystrophic epidermolysis bullosa.
Squamous cell carcinoma in junctional and dystrophic epidermolysis bullosa.
复制标题
交界处的鳞状细胞癌和营养不良性大疱性表皮松解症。
DOI：
10.1080/000155501750376285
复制
发表时间：
2001
影响因子：
3.6
通讯作者：
P. Fritsch
中科院分区：
文献类型：
--
作者：
F. Weber;J. Bauer;N. Sepp;W. Högler;W. Salmhofer;H. Hintner;P. Fritsch
我们在此报告三位隐性营养不良性大疱性表皮松解症患者及一位泛发性萎缩性良性大疱性表皮松解症患者，他们均发展为皮肤鳞状细胞癌。我们的观察和文献回顾表明，泛发性萎缩性良性大疱性表皮松解症中的鳞状细胞癌非常罕见，与隐性营养不良性大疱性表皮松解症中的皮肤癌相比，其预后更好。这些差异可以通过大疱性表皮松解症不同变种的不同病理生理和临床病程来解释。与紫外线诱导的皮肤癌不同，大疱性表皮松解症的肿瘤发生在慢性伤口愈合部位的四肢远端。本文报告的病例强调了对大疱性表皮松解症患者可疑皮肤病变进行早期组织病理学评估的特殊重要性。
We report here on three patients suffering from recessive dystrophic epidermolysis bullosa and one suffering from generalized atrophic benign epidermolysis bullosa, all of whom developed cutaneous squamous cell carcinoma. Our observations and a review of the literature suggest that squamous cell carcinoma in generalized atrophic benign epidermolysis bullosa is very infrequent and has a better outcome compared to skin cancer in recessive dystrophic epidermolysis bullosa. These differences could be explained by the distinct pathophysiology and clinical course of each of these variants of epidermolysis bullosa. In contrast to UV-induced skin cancer, the tumours in epidermolysis bullosa develop on distal extremities at sites of chronic wound healing. The cases reported here underline the exceptional importance of early histopathological assessment of suspicious skin lesions in patients with epidermolysis bullosa.