Hsieh Chih-Hsin, Huang Che-Jen, Lin Gau-Tyan
Department of Orthopaedic Surgery, Chung-Ho Memorial Hospital, Kaohsiung Medical University, No,100, Tz-you 1st road, Kaohsiung 807, ROC, Taiwan.
BMC Dermatol. 2006 Feb 15;6:2. doi: 10.1186/1471-5945-6-2.
Squamous cell carcinomas and renal failure were reported the causes of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). Death from colonic disease in epidermolysis bullosa (EB) is never reported.
We demonstrate a male patient with RDEB. He suffered megacolon due to fecal impaction and died from sigmoid colon perforation with peritonitis at age 35 years.
Constipation is a common clinical feature of RDEB, but fetal complications of chronic constipation are rarely reported. To the author's best knowledge, it has not been reported or recognized in the English literature previously. The aggressive assessment of constipation with fecal impaction is recommended in patients with RDEB.
据报道，鳞状细胞癌和肾衰竭是隐性营养不良性大疱性表皮松解症（RDEB）患者的死因。大疱性表皮松解症（EB）患者因结肠疾病死亡的情况从未有过报道。
我们展示了一名患有RDEB的男性患者。他因粪便嵌塞患上巨结肠，并于35岁时死于乙状结肠穿孔伴腹膜炎。
便秘是RDEB的常见临床特征，但慢性便秘的致命并发症鲜有报道。据作者所知，此前英文文献中尚未有过相关报道或认识。建议对RDEB患者进行积极的便秘伴粪便嵌塞评估。
