๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Introduction
Jo-David Fine and Jemima E. Mellerio Epidermolysis Bullosa 32
Synonyms:๏ก All forms of EB: EB hereditaria ๏ก EB simplex: ยญepidermolytic EB ๏ก Junctional EB: EB atrophicans, EB letalis ๏ก Dystrophic EB: EB dystrophica
Key features
ยEpidermolysis bullosa (EB) encompasses multiple clinically distinctive disorders that share three major features: genetic transmission, mechanical fragility of the skin, and blister formation
ยThere are four major forms of inherited EB โ EB simplex, junctional
EB, dystrophic EB, and Kindler EB โ which differ in the ultrastructural site within which cutaneous blisters form
ยEB may be diagnosed by immunofluorescence antigenic mapping, transmission electron microscopy, or genetic analysis
ยAny epithelial-lined or epithelial-covered organ is at potential risk for involvement in the more severe forms of inherited EB
ยIn the absence of specific therapy, management is primarily focused on prevention of blisters, wound care, and treatment of extracutaneous complications