WITHIN THE PAST 15 years, remarkable advances have been made in our understanding of each of the major types and subtypes of inherited epidermolysis bullosa (EB). These have included the more complete characterization of the breadth, severity, and relative frequency of cutaneous1 and extracutaneous2 manifestations within each EB type and subtype, estimations of the cumulative and interval risks for each known outcome or complication of the disease,3-5 determination of the prevalence and incidence of each major and minor phenotype within several geographically and ethnically distinct populations,6 and the elucidation of the molecular basis of most EB subtypes.7 Although eventually some of this knowledge may result in the successful development of beneficial gene therapies, at present there are no treatments available that appear to result in long-term reductions in blister counts or improvement in skin mechanical fragility in any form of this disease, despite numerous attempts with a variety of topical and systemically administered medications. This is particularly disappointing, since even those patients with the most localized forms of EB experience tremendous alterations in lifestyle and quality of life, and patients with some of the more generalized forms of EB are also at increased risk of early mortality.
Fine J, Eady RAJ. Tetracycline and Epidermolysis Bullosa Simplex: A New Indication for One of the Oldest and Most Widely Used Drugs in Dermatology? Arch Dermatol. 1999;135(8):981–982. doi:10.1001/archderm.135.8.981
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: