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Ocular manifestations of Junctional Epidermolysis Bullosa

RAJAKUMARI M

Abstract


Junctional epidermolysis bullosa is an inherited disorder of the basement membrane with cutaneous, ocular and other systemic manifestations. The cutaneous lesions manifest as multiple blisters from birth. Ocular features which develop from the first year of life include recurrent corneal erosions, dry eye and limbal stem cell deficiency. Risk of development of scarring corneal lesions at the age of 25 is 72.2 percentage. Hence early recognition and prompt             intervention of this disorder is of utmost importance. This is a case report of a patient who presented with advanced            features of complete stem cell deficiency.

 


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References


Cornea, Third edition, Jay H. Krachmer, MD, Mark J Mannis, MD, FACS MD, FACS and Edward J Holland, MD

American Journal of Ophthalmology 2004 Aug; 138(2): 254-62.

Eye involvement in Inherited Epidermolysis Bullosa: Experience of The National Epidermolysis Bullosa Registry

Journal of Perinatology (2004) 24, 41–47. Doi: 10.1038/Sj.Jp.7211019

www.debra.org/junctional


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