NS 3410 1st Edition Lecture 8Outline of Last LectureI. Cells in SkinII. Layers of Epidermis III. DermisIV. HypodermisV. AgingVI. Skin CancerOutline of Current LectureI. Dermal-Epidermal Junction Zone or Basement Membrane ZoneII. Epidermolysis Bullosa ComplicationsIII. Types of Inherited EBIV. Possible TreatmentsCurrent LectureI. Dermal-Epidermal Junction Zone or Basement Membrane Zone- It underlies epidermis and mucous membranes of orifices, anchors epidermis to dermis- Contains-Hemidesmosomes in basal keratinocytes-Anchoring filaments in hemidesmosomes through lamina lucida-Anchoring fibrils through lamina densa into the dermis-Focal adhesion complex binds actin cytoskeleton to basement membraneII. Epidermolysis Bullosa ComplicationsThese notes represent a detailed interpretation of the professor’s lecture. GradeBuddy is best used as a supplement to your own notes, not as a substitute.- Infection: blistering skin is vulnerable to bacterial infection- Sepsis: Occurs when bacteria from a massive infection enters bloodstream and spreads rapidly throughout the body. Rapidly progressing, life-threatening condition- Deformities: severe forms of EB can cause fusion of fingers or toes and abnormal bending of joints- Malnutrition and anemia: blisters in mouth can make eating difficult and lead to malnutrition, which may lead to anemia, delayed would healing, slow growth- Dehydration: large, open blisters can cause loss of body fluids that leads to severe dehydration- Constipation: difficulty passing stool may be due to painful blisters in anal area and by not ingesting enough liquids or high fiber foods- Eye disorders: inflammation of eye can harm clear covering of eye- Skin cancer: high risk of developing squamous cell carcinoma- Death: infants with a severe form of junctional EB are at high risk of infections and loss of body fluids from widespread blistering; their survival may also be threatened because of blistering, which may hamper their ability to eat and breathe- Water loss from skin: damage to epithelia increases insensible perspirations (water lost from interstitial fluids due to evaporation); when connections between the superficial and deep layers of the epidermis are damaged, fluid accumulates in pockets or blisters within the epidermis or can form between the epidermis and dermis if basement membrane is damaged-Get dangerous fluid loss/xerosis= excessively dry skinIII. Types of Inherited EB- Common complications associated with inherited EB:-Skin blistering-Skin heals with atrophy and hypopigmentation-Alopecia= hair loss-Progressive nail dystrophy and loss-Increased dental caries-Early loss of teeth-Lung disease-Congenital nephrotic syndrome-Contractures= shortening of muscular or connective tissue-Dysphagia-Skin Cancer- Simple-Apparent at birth or during early infancy-Most common and least severe type-Blistering may be relatively mild-Due to mutation in Keratin Intermediate filaments- Junctional-Becomes apparent at birth and is usually severe-Large, ulcerated blisters lead to infections and loss of body fluids-May be fatal-Mutations in hemidesmosomal components - Dystrophic -Apparent at infancy or birth-Rough, thickened skin, scarring and disfigurement of hands and feet-Mutations in COL7A1- Kindler SyndromeIV. Possible Treatments- Gene therapy: replace mutant gene with functional gene- Protein replacement therapy: recombinant protein- Cell Therapy- Culturing/Grafting: acquire patches of skin that have C7 and anchoring