Inherited blistering diseases of the skin.
Inherited epidermolysis bullosa (EB) comprises some 23 different phenotypic disorders which have as common features the presence of mechanically fragile skin and the tendency to develop blisters, erosions, and in some cases, scarring of the skin. While some forms of this disease are relatively mild, other rarer variants may prove to be life-threatening. In the past 15 years, a wealth of new information has elucidated new clinical and laboratory characteristics of specific EB subsets. In this brief review, the most important aspects of inherited EB are summarized to update the practicing pediatrician about the ever-increasing complexities being discovered about this disease.
Fine
J D
JD
Department of Dermatology, School of Medicine, University of North Carolina, Chapel Hill.
Johnson
L B
LB
Wright
J T
JT
D001485:Basement Membrane; D016108:Epidermolysis Bullosa Dystrophica / Q000175:diagnosis / Q000209:etiology / Q000235:genetics / Q000628:therapy; D016110:Epidermolysis Bullosa Simplex / Q000175:diagnosis / Q000209:etiology / Q000235:genetics / Q000628:therapy; D016109:Epidermolysis Bullosa, Junctional / Q000175:diagnosis / Q000209:etiology / Q000235:genetics / Q000628:therapy; D006801:Humans; D011379:Prognosis