Two Cases of Dominant Dystrophic Epidermolysis Bullosa.
- Author:
Chang Kyun LEE
1
;
Seong Jun SEO
;
Chang Kwun HONG
;
Byung In RO
;
Kye Yong SONG
;
Ju Huep LEE
Author Information
1. Department of Dermatology, College of Medicine, Chung Ang Univerisity, Korea.
- Publication Type:Case Report
- Keywords:
Dominant dyst ophic epidermolysis bullosa;
Family history
- MeSH:
Ankle;
Basement Membrane;
Cicatrix;
Elbow;
Epidermolysis Bullosa Dystrophica*;
Hand;
Humans;
Knee;
Microscopy, Electron;
Parturition
- From:Korean Journal of Dermatology
1997;35(4):758-762
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
