JEB Inversa, is a late onset of JEB
and Larygo-onycho-cutaneous syndrome are caused by mutations in the laminin-332
gene. It is presents at birth with blisters localized to intertriginous areas
such as groin, neck and axillae. although Milia are not common, scarring and
nail dystrophy frequently are observed. Oral blister my occur, and dental
enamel Hypoplasis and dental caries may be more common. With the exception of Pyloric atresia, which may occur, other
systemic findings such as poor growth, anemia, and blistering and other
problems involving the trachea/respiratory tract, genitourinary tract and eyes
are not seen.
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