Herlitz Junctional EB

       Herlitz Junctional EB is the most severe subtype of JEB. It is caused by mutations in the laminin gene. Affected people generally present at birth with severe, widespread blisters, although, in some cases, blistering at birth may be mild. After someone is born with Herlitz JEB the skin begins to get more blister and more sores.  They can get sore around the diaper area, nose and mouth and on the nail beds and the fingertips and toes, on the scalp and on the neck.

          Poor dental enamel, oral erosions and dental caries are common. Poor growth, anemia, and blistering and other problems involving the trachea/respiratory tract, intestines/gastrointestinal tract, eyes or genitourinary tract are common. Laryngeal blisters and resultant scars cause a hoarse cry during infancy. Tracheal blistering and scarring may cause occlusion of the trachea and suffocation if not recognized and treated.