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Epidermolysis bullosa
- 2. Structure of Basement membrane or Dermoepidermal Junction Constituent • Keratin filament • Hemidesmosomes (Plectin, Integrin, Dystonin or BPAg1- Bullous pemphigoid antigen 1) • Lamina Lucida or Anchoring filaments (Laminin and BPAg2) • Lamina Densa (Collagen IV) • Lamina Fibroreticularis (Collagen VII) Keratin 5 & 14 Hemidesmosomes Lamina Lucida Lamina Densa Lamina Fibroreticularis
- 3. Epidermolysis Bullosa Heterogenous group of disorder characterize by tendency to blister on slight trauma Classification on the basis of split in basement membrane Variant Inheritance Gene defect Level of split EB Simplex ADI Keratin 5 & 14 Basal Area Dominant Dystrophic ADI Collagen IV Below Lamina Densa Junctional EB ARI Laminin Lamina Lucida Recessive Dystrophic ARI Collagen VII Below Lamina Densa
- 4. Epidermolysis Bullosa Simplex EB Simplex Features Inheritance ADI Onset Early childhood Bullae Non-hemorrhagic Healing Without scarring Distribution Hands and feet spare Nails and mucosa
- 5. Dominant Dystrophic Epidermolysis Bullosa D. Dystrophic EB Features Inheritance ADI Onset At birth or Infancy Bullae Hemorrhagic Healing Scarring & Milia Distribution Site of trauma Involves Nails Spare Mucosa
- 6. Junctional Epidermolysis Bullosa or EB Letalis Junctional EB Features Inheritance ARI (Rare) Onset At birth Bullae Large & Flaccid Healing Scarring & Milia Distribution Generalized then Perioral & perianal Involves Nails Loss & Mucosa
- 7. Recessive Dystrophic Epidermolysis Bullosa R. Dystrophic EB Features Inheritance ARI Onset At birth Bullae Hemorrhagic Healing Severe Scarring & Milia Distribution Site of trauma Involves Nails and Mucosa Complications Webbing of digits, esophageal strictures Skin Cancer (SCC)
- 8. Investigations Transmission electron microscopy (confirms level of bulla) Treatment Counselling Avoid trauma Wear soft shoes and clothes Keep feet dry In presence of blister- prick it without removing the roof Non-adhesive dressing Gene therapy Surgery foe mitten hands, strictures etc.