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Open Access Case report

Death from colonic disease in epidermolysis bullosa dystrophica

Chih-Hsin Hsieh2, Che-Jen Huang3 and Gau-Tyan Lin1*

Author Affiliations

1 Department of Orthopaedic Surgery, Chung-Ho Memorial Hospital, Kaohsiung Medical University, No.100, Tz-you 1st road, Kaohsiung 807, R.O.C, Taiwan

2 Department of Emergency, Chung-Ho Memorial Hospital, Kaohsiung Medical University, No.100, Tz-you 1st road, Kaohsiung 807, R.O.C, Taiwan

3 Division of Gastroenterology and General Surgery, Chung-Ho Memorial Hospital, Kaohsiung Medical University, No.100, Tz-you 1st road, Kaohsiung 807, R.O.C, Taiwan

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BMC Dermatology 2006, 6:2  doi:10.1186/1471-5945-6-2

Published: 15 February 2006

Abstract

Background

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.

Case presentation

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.

Conclusion

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.