Epidermolysis bullosa dystrophica inversa: A case report
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Enver Turan 1 * , Mehmet Salih Gürel, Aslı Turgut Erdemir, Burcu Işık, Nurdan Yurt
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1 Harran University, Faculty of Medicine, Department of Dermatology Şanlıurfa, Turkey* Corresponding Author

Abstract

Epidermolysis bullosa (EB) is a heterogeneous group of genetically determined, mechanobullous disorders characterized by blister formation in response to mechanical trauma. Three major subgroups, simplex, junctional, and dystrophic EB, contain more than 20 genetically and clinically distinct subtypes. Epidermolysis bullosa dystrophica inversa (DEB-I), which is a rarely seen form of epidermolysis bullosa, shows autosomal recessive inheritance and it is characterized by bulla formation and erosions on flexural areas, frequently affecting the oral and esophageal mucosa. Blistering rash occurs in the newborn period, which in early childhood heals with atrophic scars. DEB-I is rarely seen in adults period, with only a few case reports in the literature. In the present case, we described a patient diagnosed with epidermolysis bullosa dystrophica inversa due to presence of typical clinical features and histopathological and immunofluorescence findings.

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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Article Type: Case Report

J Clin Exp Invest, 2012, Volume 3, Issue 3, 412-414

https://doi.org/10.5799/ahinjs.01.2012.03.0191

Publication date: 11 Sep 2012

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