EB Simplex-Ogna is a rare autosmal dominant form of EB simplex. it is due by a mutation in the plection gene. Blistering at birth that predominantly involves the hand and feet. More generalized blistering also may develop. milia and scarring typically do not occur.
The characteristic features of EBS-Ogna is the development of very thickened nails,temed onychogryphosis, as well as tendency to bruise. dental enamel is normal. Blistering of the mouth does not occur.
Affected people typically don not develop poor growth, anemia, or problems with the trachea/respiratory tract, intestines/gastrointestinal trace, eyes or genitourinary tract.
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