Gayathri E, Anbukkarasi K, Lilly S Mary
Department of Pathology, Sree Balaji Medical College and Hospital, Bharath University, Chennai, Tamil Nadu, India.
Indian J Pathol Microbiol. 2023 Apr-Jun;66(2):360-362. doi: 10.4103/ijpm.ijpm_249_21.
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.
营养不良性大疱性表皮松解症（EBD）是一种真皮上层结构蛋白的遗传性疾病，其特征是在创伤部位形成水疱，随后出现瘢痕形成。皮肤脆弱和水疱形成是这种疾病的标志。皮肤鳞状细胞癌（cSCC）是大疱性表皮松解症（EB）患者中一种可怕的并发症，也是常见的死亡原因。不同肿瘤微环境的最新进展解释了隐性营养不良性大疱性表皮松解症（RDEB）患者中鳞状细胞癌的侵袭性本质，以及使用VII型胶原蛋白重新表达作为一种可能的治疗措施。定期随访对于预防并发症至关重要。
