Wednesday, October 17, 2012

Mortality...


Infancy is an especially difficult time for Epidermolysis Bullosa (EB) patients. Generalized blistering caused by any subtype may be complicated by infection, sepsis, and death. Severe forms of Epidermolysis Bullosa (EB) increase the mortality risk during infancy. Patients with the Herlitz or letalis form of Junctional Epidermolysis Bullosa [JEB] have the highest risk during infancy with an estimated mortality rate of 87% during the first year of life. The patients with Epidermolysis Bullosa (EB) who survive childhood, the most common cause of death for them is metastatic squamous cell carcinoma (SCC):


Recessively inherited dystrophic Epidermolysis Bullosa (EB), squamous cell carcinoma (SCC)

This skin cancer occurs specifically in patients with recessively inherited Epidermolysis Bullosa (EB) who most commonly are aged 15-35 years. In contrast, dominantly inherited Epidermolysis Bullosa Simplex [EBS] and Dystrophic Epidermolysis Bullosa [DEB] and milder forms of Junctional Epidermolysis Bullosa [JEB] may not affect a patient's life expectancy adversely.

One study reported that from 1979-2002, the overall age-adjusted annual mortality annual mortality rate from bullous skin diseases 0.103 death per 100,000 population (2000 US standard population).



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