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.
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