营养不良性大疱性表皮松解
大疱性表皮松解症
医学
皮肤病科
基底细胞
真皮
病理
疾病
作者
E Gayathri,K. Anbukkarasi,S Mary Lilly
出处
期刊:PubMed
日期:2023-04-20
卷期号:66 (2): 360-362
标识
DOI:10.4103/ijpm.ijpm_249_21
摘要
Epidermolysis bullosa dystrophica (EBD) is an inherited disease of the structural proteins in the upper dermis, characterized by blister formation at the site of trauma followed by scarring. Skin fragility and blistering are the hallmarks of this disease. Cutaneous squamous cell carcinoma (cSCC) is a dreadful complication in the epidermolysis bullosa (EB) patients and common cause of death. The recent advances in distinct tumor microenvironment explain the aggressive nature of SCC in recessive Recessive Dystrophic Epidermolysis Bullosa (RDEB) patients and the use of collagen VII re-expression as a possible therapeutic measure. Regular follow-up is a must in preventing complications.
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