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.
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| http://dx.doi.org/10.33699/PIS.2022.101.2.90-93 | DOI Listing |
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Centro de Ciências Naturais e Humanas, Universidade Federal do ABC, Av. dos Estados, 5001. Bairro Bangu, Santo André, SP, 09210-580, Brazil.
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