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Ileus as a complication of butterfly wing disease − case report


Authors: P. Guňková 1;  D. Toman 1;  T. Posolda 2
Authors‘ workplace: Chirurgická klinika Fakultní nemocnice Ostrava 1;  Oddělení centrálních operačních sálů Fakultní nemocnice Ostrava 2
Published in: Rozhl. Chir., 2022, roč. 101, č. 2, s. 90-93.
Category: Case Report
doi: https://doi.org/10.33699/PIS.2022.101.2.90–93

Overview

Epidermolysis bullosa or butterfly wing disease is a rare genetic disorder of connective tissue associated with the formation of blisters. The clinical manifestations are very diverse and affect not only the skin cover, but also mucosal surfaces of the gastrointestinal, urogenital and respiratory systems. A large proportion of patients suffer from severe constipation with the development of megacolon. The case report describes intestinal obstruction in a patient with a dystrophic form of epidermolysis bullosa which required surgical treatment.

Keywords:

epidermolysis bullosa – bowel obstruction


Sources

1. Freeman EB, Koglmeier J, Martinez AE, et al. Gastrointestinal complication of epidermolysis bullosa in children. Br J Dermatol. 2008;158:1308−1314. doi:10.1111 /j.1365-2133.2008.08507.

2. Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa. Part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61:367−384. doi:10.1016/j. jaad.2009.03.052.

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4. Hsieh CH, Huang CJ, Lin GT. Death from colonic disease in epidermolysis bullosa dystrophica. BMC Dermatol. 2006;6(2). doi:10.1186/1471-5945-6-2.

5. Horn HM, Tidman MJ. The clinical spectrum of dystrophic epidermolysis bullosa. Br J Dermatol. 2002;146(2):267−274. doi:10.1046 /j.1365-2133.2002.04607.

6. Kralik J, Rapant V. Radical surgical treatment of esophageal stenosis due to epidermolysis bullosa. J Thorac Cardiovasc Surg. 1975;69(5):790−792. PMID:1127977.

7. Smith PK, Davidson GP, Moore L, et al. Epidermolysis bullosa and severe ulcerative colitis in an infant. The Journal of Pediatrics 1993;122(4):600−603. doi:10.1016/ s0022-3476(05)83545-0.

8. Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa. Part II. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):387–402. doi: 10.1016/j.jaad.2009.03.053.

Labels
Surgery Orthopaedics Trauma surgery
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