Dystrophic epidermolysis bullosa(DEB) is a rare, chronic non-inflamrnatory bullous disease of hereditary trait, which easily produces bullae in response to even minor traurna. We report two cases which developed at birth and infancy, respectively. Bullae, atrophic scars on the knees, ankles and elbows, as well as milia of both hands and loss of nails were seen in both cases. Histologically, cleavage occurs beneath the basal lamina and anchoring fibrils are rudirnentary on electron microscopy. We present two cases of DEB that seem to be dominant types considering the presence of family history with clinical, histological and electronmicroscopic findings.
Ankle ; Basement Membrane ; Cicatrix ; Elbow ; Epidermolysis Bullosa Dystrophica* ; Hand ; Humans ; Knee ; Microscopy, Electron ; Parturition