Epidermolysis bullosa (EB) is a rare heritable disease clasjficated to some types. Dystrophic Epidermolysis bullosa (DEB) is an severe one, which has many complications causing death. Aims: To describe clinical signs and symptoms, lab findings as well as complications of DEB. Subject and method: Exploring 14 cases diagnosed DEB in the National Hospital of Pediatrics from Oct. 2010 to Oct. 2012. Result: Gender: male/female = 1:1. The average ages: 7 + or - 4.9. Clinical features: 100 percent of patients appear blisters, bullosa, exfoliation when they have a slight collision right after birth
oral and anal lesions are 100 percent
eye lesions 64.3 percent
abnormal nail 100 percent including 64.3 percent with no nails and 35.7 percent dystrophic nail
and dental lesions 100 percent . Investigations: 85.7 percent of patients have hypochromic microcytic anemia, moderate Hb 8.56 + or - 2.45 g/l. Skin biopsy and histopathological analysing by 3 techniques HE dyeing, electronic microscope, immunofluorescence showed that above and below the membrane of the skin, the collagen VII was absent or just the faint images. Complications: 1 patient died because of septicemia, accounting for 0.7 percent
malnutrition 92.9 percent
skin scars 100 percent
stick the toes or fingers 78.6 percent
constipation 85.7 percent
and 100 percent of patients have pains and itching which affected sleeping. Conclusions: DEB is characterized by blisters in the skin and mucosal membranes which develop in response to minor injury, appeared soon after birth
the typical pathology images are the lack of collagen VII. The severe complications of physical and mental have serious impacts on patients' quality of life.