Non-herlitz junctional epidermolysis bullosa.

[junctional epidermolysis bullosa]

Non-Herlitz junctional epidermolysis bullosa (nH JEB ) is characterized by generalized blisters that predominate in sites exposed to friction , trauma , or heat . Whereas infants and children with nH JEB often appear to resemble patients with other forms of EB , adults with this disorder typically display atrophic scars , hypopigmentation , or hyperpigmentation at sites of healed blisters as well as incomplete alopecia , dystrophic nails , mucous membrane involvement , and dental abnormalities . Mild (or severe ) disease early in life may be characterized by the opposite phenotype in adults with nH JEB . Although nH JEB is generally less severe than Herlitz disease , fatalities (especially in neonates ) are not uncommon among patients with the former diagnosis .