Non-Herlitz Junctional EB

Non-Herlitz Junctional EB (JEB-nH) generally is less severe then Herlitz JEB, and there are two main types: Generalized non-Herlitz JEB, which may be caused y mutations in the collagen XVII gene or the laminin -332 gene. Localized non- Herlitz JEB, which is caused by mutations in the collagen type XVII gene.

          Generalized Non-Herlitz JEB is presents at birth with widespread blistering and erosions. Blistering usually is worse in periods of warm, humid weather. Milia scarring and nail dystrophy are common and mild thickening of the skin on the palms and soles my occur. Blistering on the scalp is common, and permanent hair loss may occur.

          Poor dental enamel, oral erosions and dental caries are common, often necessitating extensive dental reconstruction. you can have poor growth, anemia, and blistering and other problems that could involving the trachea/respiratory tract, intense/gastrointestinal tract.

          you could get blisters in your larynx which could cause scars and a hoarse cry during infancy, and a hoarse, husky voice may remain through adulthood. Tracheal blisters may occur in individuals with JEB-nH, and, if they occur when the affected person is an infant with a relatively small trachea, they may cause occlusion of the trachea and suffocation if not recognized and treated.