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International Journal of Health and Rehabilitation Sciences

International Journal of Health and Rehabilitation Sciences
ISSN (online) : 2278-9030
ISSN (Print) : 2278-9014
ISSUES : 4/year
PUBLISHER : Rehabilitation Without Borders

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Case Report 
IJHRS. 2013; 2(3): 165-172


Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review

S. Karthika Nagarajan, Pushparaja Shetty.

Abstract
Aim: The paper aims to present a unique case of Epidermolysis bullosa (EB) with oral manifestations. The paper also discusses briefly the latest concepts in management of EB patients in a Dental setup.

Observations: A 25 years old girl with a history of blister formation over the abdomen at 4 days of age that gradually presented all over the body by the age of 6 months and present till date. Bullae subsequently broke-down to form ulcers which healed gradually leaving scars. Multiple milias were observed over the face. Extensive cicatricial alopecia present over the occipital region of the scalp (15 X 20 cms). Fingers and toes exhibited nail dystrophy. Patient reported of hearing difficulty and has been diagnosed as suffering from Mitral regurgitation and Pulmonary arterial hypertension. Extra and Intraoral examination revealed Microstomia, Hypoplastic teeth, Chronic generalized Periodontitis with severe bone loss. Histopathological examination of bullae from oral mucosa using Pap stain revealed a Subepidermal split. The case was diagnosed as rare form of Generalised Recessive Dystrophic Epidermolysis Bullosa (RDEB).

Conclusion: Epidermolysis bullosa is one among the Dermatological diseases having classical findings in the Orofacial region along with other general manifestations. Dental surgeons need to be aware of such conditions in their patients to instigate appropriate treatment procedures. Multidisciplinary approach and priority treatment needs are set by the current working groups on EB that may guide our approach to patient management.

Key words: Epidermolysis Bullosa, Cicatricial alopecia, Milia, Microstomia, Mitral regurgitation


 
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How to Cite this Article
Pubmed Style

S. Karthika Nagarajan, Pushparaja Shetty. Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. IJHRS. 2013; 2(3): 165-172.


Web Style

S. Karthika Nagarajan, Pushparaja Shetty. Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. http://www.ijhrs.org/?mno=46272 [Access: October 17, 2021].


AMA (American Medical Association) Style

S. Karthika Nagarajan, Pushparaja Shetty. Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. IJHRS. 2013; 2(3): 165-172.



Vancouver/ICMJE Style

S. Karthika Nagarajan, Pushparaja Shetty. Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. IJHRS. (2013), [cited October 17, 2021]; 2(3): 165-172.



Harvard Style

S. Karthika Nagarajan, Pushparaja Shetty (2013) Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. IJHRS, 2 (3), 165-172.



Turabian Style

S. Karthika Nagarajan, Pushparaja Shetty. 2013. Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. International Journal of Health and Rehabilitation Sciences (IJHRS), 2 (3), 165-172.



Chicago Style

S. Karthika Nagarajan, Pushparaja Shetty. "Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review." International Journal of Health and Rehabilitation Sciences (IJHRS) 2 (2013), 165-172.



MLA (The Modern Language Association) Style

S. Karthika Nagarajan, Pushparaja Shetty. "Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review." International Journal of Health and Rehabilitation Sciences (IJHRS) 2.3 (2013), 165-172. Print.



APA (American Psychological Association) Style

S. Karthika Nagarajan, Pushparaja Shetty (2013) Epidermolysis Bullosa associated with Enamel Hypoplasia, Multiple Missing Teeth and Mitral Regurgitation A Case Review. International Journal of Health and Rehabilitation Sciences (IJHRS), 2 (3), 165-172.



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