Epidermolysis Bullosa Dystrophica
نویسندگان
چکیده
منابع مشابه
Anesthetic implications in epidermolysis bullosa dystrophica.
Epidermolysis bullosa is a genetic mechanobullous disease of the stratified squamous keratinizing epithelium that affects the skin and mucous membranes. Its primary feature is the formation of blisters after minor shearing trauma to the skin or mucous membranes that can result in debilitating, even life-threatening scarring. The disease presents special problems for the anesthesia provider beca...
متن کاملClinical Management for Epidermolysis Bullosa Dystrophica
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are p...
متن کاملAcquired double-barrel oesophagus in epidermolysis bullosa dystrophica.
An unusual case of epidermolysis bullosa dystrophica with extensive stenosis, high perforation, and dissection of the oesophagus forming a "double-barrel" structure is described. Gastric epithelium found in the upper oesophagus is thought to be of metaplastic origin and caused by repeated minor trauma with repair.
متن کاملDeath from colonic disease in epidermolysis bullosa dystrophica
BACKGROUND Squamous cell carcinomas and renal failure were reported the causes of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). Death from colonic disease in epidermolysis bullosa (EB) is never reported. CASE PRESENTATION We demonstrate a male patient with RDEB. He suffered megacolon due to fecal impaction and died from sigmoid colon perforation with peritonitis at...
متن کاملManagement of oesophageal stenosis in epidermolysis bullosa dystrophica.
Seven patients with epidermolysis bullosa dystrophica and chronic and recurrent oesophageal lesions such as spasm, strictures, and complete occlusion were studied. Dysphagia could be cured with drugs if it was caused by bullae formation or spasm. If oesophageal strictures were present, endoscopy and bouginage with corticosteroid prophylaxis during the quiescent phase of the disease was a safe a...
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ژورنال
عنوان ژورنال: Proceedings of the Royal Society of Medicine
سال: 1947
ISSN: 0035-9157
DOI: 10.1177/003591574704001211