Dystrophic Epidermolysis Bullosa Syndrome

Objective In order to improve medical doctors’cognition and standard of diagnosis of Inherited dystrophic epidermolysis bullosa syndrome,and decrease misdiagnosis.Methods Analyze and summarize the patients’ clinical medical record, discuss the clinical characteristics, histopathologic, diagnosis, differential diagnosis and treatments according to associated references.Results Blisters and bullae were discovered around the whole body when the patient was born;Histopathological of blisters on he right arm found that the blisters is located below the epidermis;Immunofluorescence staining shows the expression level of the basement membrane is reduced. Electron microscopic image of dystrophic epidermolysis bullosa shows dissociation immediately below the lamina densa.Along the dermal-epidermal junction in the dystrophic epidermolysis bullosa patient’s skin, the hemidesmosomes appear reduced or abnormal.And excluding the differential diagnosis which have the similar clinical manifestations,the diagnosis of inherited dystrophic epidermolysis bullosa syndrome was made.Conclusion Inherited dystrophic epidermolysis bullosa syndrome (DEB ) is rare in clinic,easy to misdiagnose. In the clinical treatment process, we can combine with the the clinical characteristics, histopathology, immunofluorescence, Transmission Electron Microscope to made exact diagnosis and the diagnosis of type.