Epidermolysis bullosa Dystrophica is rare, chronic non-inflammatory- mechanobullous disease of hereditary trait, which easily produces bulla by minor trauma or spontaneously.
The lesions usually appears from birth or in infant involving especially lower leg, hand, foot and leads to erosive, hemorrhagic bulla, secondary infection, pruritus and scar formation and- sometimes deformity of nails and cicatrical alopecia. It sometimes involves oral mucosa, esophagus, eye, tooth, bone, respiratory system, anus and vagina. This disease has rare tendency for carcinogenesis from cicatrical lesions. These manifestations are more severe in recessive type than in dominant type.
We had observed 5 cases of EBD at Ewha Womans University Hospital from Sept.. 1975. to Jul. 1976.
Among 5 cases, 4 cases occurred from birth, 1 case occurred at age 1. Skin lesions. were hand, foot, knee area in all 5 cases. Head, face were involved in 2 cases. Arm, leg, trunk, buttock were involved in 3 cases. In skin manifestations,. bulla, erosive bulla, secondary infection, scar formation, pruritus, nail deformity, positive Nikolsky sign were observed in all 5 cases. Hemorrhagic bulla, miliaria were presented. in 1 case, alopecia in 2 cases. Aggrevated season were summer in 4 cases, spring in 1 case. Histologically, microscopic observation, after H-E stain, revealed hyperkeratosis in 3 cases, acanthosis in 2 cases, partial atrophy in 1 case,, partial ulcer in 1 case, snbepidermal bulla in 5 cases, chronic nonspecific inflammatory cell infiltration of upper dermis in 3 cases, fibrosis of lower dermis in 1 case.
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