Ye X, Warrier S, Nejad K J, Parasyn A D
Prince of Wales Clinical School, The University of New South Wales, Australia.
J Surg Case Rep. 2012 Aug 1;2012(8):10. doi: 10.1093/jscr/2012.8.10.
Epidermolysis Bullosa (EB) is a complex group of genetic disorders characterised by mechanical fragility in the basement membrane zone. Affected individuals experience significant morbidity and mortality, most commonly from cutaneous malignancies. In fact, 90.1% of EB patients develop Squamous Cell Carcinoma (SCC) before the age of 55, 80% of whom die within 5 years of diagnosis. Furthermore, the management of cutaneous malignancies in EB is fraught with challenges given the atypical presentations of malignancies and the co-existence of systemic co-morbidities. To illustrate the common pearls and pitfalls of managing EB in the perioperative setting, we present a case of SCC of the left forefoot which spread through a natural plane of weakness in the dermal-epidermal junction as a complication of the congenital weakness in the area resulting from EB.
大疱性表皮松解症(EB)是一组复杂的遗传性疾病,其特征是基底膜区存在机械性脆弱性。受影响的个体经历显著的发病率和死亡率,最常见的死因是皮肤恶性肿瘤。事实上,90.1%的EB患者在55岁之前会发生鳞状细胞癌(SCC),其中80%在诊断后5年内死亡。此外,鉴于恶性肿瘤的非典型表现以及全身性合并症的共存,EB患者皮肤恶性肿瘤的管理充满挑战。为了阐述围手术期管理EB的常见要点和陷阱,我们呈现了一例左前足SCC病例,该病例通过皮肤-表皮交界处的天然薄弱平面扩散,是EB导致该区域先天性薄弱的并发症。