| Literature DB >> 19945618 |
Kim B Yancey1, Helmut Hintner.
Abstract
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.Entities:
Mesh:
Year: 2010 PMID: 19945618 DOI: 10.1016/j.det.2009.10.008
Source DB: PubMed Journal: Dermatol Clin ISSN: 0733-8635 Impact factor: 3.478