Bart’s Syndrome: A Case Report

International Journal of Medical Science

© 2015 by SSRG - IJMS Journal

Volume 2 Issue 9

Year of Publication : 2015

Authors : V.V.V. Satyanarayana

10.14445/23939117/IJMS-V2I9P101

How to Cite?

V.V.V. Satyanarayana, "Bart’s Syndrome: A Case Report," SSRG International Journal of Medical Science, vol. 2,  no. 9, pp. 1-6, 2015. Crossref, https://doi.org/10.14445/23939117/IJMS-V2I9P101

Abstract:

Bart syndrome is a genetic disorder characterized by the association of congenital localized absence of skin(CLAS), epidermolysis bullosa (EB) , lesions of the mouth mucosa, and dystrophic nails. It may be associated with any type of EB but is mostly reported with dominant dystrophic EB (DEB dominant).Clinically it is characterized by raw beefy areas of denuded skin on trauma prone areas of body. E.g. Hands & Feet. Diagnosis is obvious clinically but it requires ultra structural microscopy for proper classification of the disease. Treatment is only palliative measures. Here we report a case; A mother gave birth to two female child with 3 years gap. Both sisters showed clinical features of Bart’s syndrome. 1 st child Complaining of scars on both legs and bulla and 2nd child complaining of red-rich area and absence of skin on Right leg and foot with hemorragic bulla. We report these cases because of its rare disorder.

Keywords:

Bart’s Syndrome, EB, dominant DEB, Congenital localized absence of skin, Genetic Mechanobullous disorder.

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