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Vesiculobullous diseases-1

Dystrophic epidermolysis bullosa both autosomal

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Unformatted text preview: YSIS 3. Dystrophic Epidermolysis Bullosa Both autosomal dominant and recessive; recessive is Both severe severe Lesions are birth; arise at pressure sites Blisters rupture leaving painful ulcers which heal with Blisters large scars that undergo contractures, leading to loss of motility and claw-like hands (Mitten Deformity) motility Teeth exhibit delayed eruption and enamel hypoplasia Teeth with rapid caries development with Scarring around mouth leads to diminished opening, Scarring ankyloglossia ankyloglossia EPIDERMOLYSIS BULLOSA EPIDERMOLYSIS Epidermolysis Bullosa Acquisita Non-hereditary form; appears in adulthood Clinically resembles autosomal dominant Clinically type of JEB-type VII collagen type Trauma/friction induced blisters of knees, Trauma/friction elbows, hands and feet- heal with scars elbows, Intraoral blisters rare- when present same Intraoral picture same picture as JEB picture EPIDERMOLYSIS BULLOSA EPIDERMOLYSIS HISTOPATHOLOGY Simplex type exhibits zone of cleavage Simplex (intra-epithelial) above basal cell layer. (intra-epithelial) Remaining types have sub-epithelial Remaining separation separation EPIDERMOLYSIS BULLOSA EPIDERMOLYSIS EPIDERMOLYSIS BULLOSA EPIDERMOLYSIS MANAGEMENT No specific treatment available for hereditary No types types Acquired form maybe treated with Acquired corticosteroids and immuno-suppressants corticosteroids Maintenance of pt’s nutritional and oral hygiene Maintenance status status Wound healing techniques Prevention of infections Systemic use of Phenytoin (also acts as a Systemic collagenase inhibitor) collagenase...
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