Peter D. Witt, MD; Daniel T. Cohen, BA; Susan B. Mallory, MD; from the Departments of Surgery, Plastic and Reconstructive (Drs Witt and Mr Cohen), Internal Medicine (Dermatology) (Dr Mallory), and Pediatrics (Dr Mallory), Washington University School of Medicine, St Louis Children’s Hospital, and the Department of Plastic and Reconstructive Surgery, Shriners Hospital for Children (Dr Witt), St ...

Epidermolysis bullosa (EB) is a group of inherited blistering skin diseases that vary widely in their pathogenesis and severity. It has been divided into distinct subtypes depending on the level of tissue separation in the dermal- epidermal basement membrane zone. There are four main categories of EB: simplex, junctional, dystrophic and Kindler syndrome. Junctional epidermolysis bullosa with py...

In dystrophic epidermolysis bullosa the genetic defect of anchoring fibrils leads to cleavage beneath the basement membrane, with its consequent loss. We performed scanning electron microscopy of an inverted blister roof of a case of dystrophic epidermolysis bullosa, confirmed by immunomapping and gene sequencing. With a magnification of 2000 times a net attached to the blister roof could be ea...

Marsden, R. A., Sambrook Gowar, F. J., MacDonald, A. F., and Main, R. A. (1974). Thorax, 29, 287-295. Epidermolysis buBosa of the oesophagus with oesophageal web formation. Four members of a family are described with epidermolysis bullosa dystrophica of probable autosomal recessive inheritance. They have shown the typical blistering of the skin and oral mucosa, usually present at birth, which o...

in this paper, two patients with severe dysphagia and esophageal stricture secondary to epidermolysis bullosa are presented and discussed. they are siblings of an affected family. primary resection and anastomosis had been performed previously in both patients, but dysphagia had recurred. we treated these patients with free jejunal graft to the esophagus with excellent results. both patients ar...

context connective tissue cells include fibroblasts, chondrocytes, adipocyte, and osteocytes. these cells are specialized for the secretion of collagenous extracellular matrix and are responsible for the architectural framework of the human body. evidence acquisition connective tissue cells play a central role in supporting as well as repairing tissues and organs. fibroblast cell therapy could ...

Epidermolysis bullosa rarely affects lower airways. We present a case of lower airway involvement and stenosis successfully managed with flexible bronchoscopy and balloon dilation.