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Dermatology and Infectious Diseases. Disorders of Keratinisation Dermatitis Blistering Disorders Immunology. Disorders of Keratinisation. Overview Psoriasis Icthyosis. Objectives. Disorders of Keratinisation. Disorders of Keratinization. Keratinization
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Dermatology and Infectious Diseases Disorders of Keratinisation Dermatitis Blistering Disorders Immunology
Disorders of Keratinisation • Overview • Psoriasis • Icthyosis
Objectives • Disorders of Keratinisation
Disorders of Keratinization Keratinization Terminal differentiation of epithelia • epithelial proteins (Keratin) • Glycoproteins (Cell Envelope) • intercellular lipids
Disorders of Keratinization • Cause changes in the skin • Dry, Scaly, Thickened, Flaky • Blistering • Cause changes in Mucous membranes, Nails and Hair
Disorders of Keratinization • Change in Type of Keratin Made
Disorders of Keratinization • Psoriasis • Icthyosis
Disorders of KeratinizationPsoriasis • Chronic , relapsing and remitting skin disease. • May appear at any age • may affect any part of the skin • Common Locations: • Extensor surfaces Knees and Elbows
Disorders of KeratinizationPsoriasis • Characterised by hyperproliferation of skin and inflammation
Disorders of KeratinizationPsoriasis • Etiology • Inherited • Abnormality of Ca++ metabolism • Genetic Predisposition • HLA Cw6 • HLA DR7 • HLA B27 (Pustular)
Disorders of KeratinizationPsoriasis • Etiology • Environmental Factors • Stress, Smoking and Alcohol • Systemic Drugs • Infection • Immunological Factors
Psoriasis Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)
Disorders of KeratinizationPsoriasis • Abnormalities in Psoriatic Skin • transit time through epidermis • mitotic activity • rate of DNA synthesis • high levels of Ca++ binding protein calmodulin • Presence of keratin 6 & 16 in epidermis • These five are must knows
Disorders of KeratinizationPsoriasis • Abnormalities in Psoriatic Skin • levels of phospholipase A2 activity • levels of polyamine synthesis • levels of plasminogen activator • cGMP levels leading to a high ratio cGMP/cAMP • These 4 are included for the sake of completeness
Disorders of KeratinizationPsoriasis • Pathology • Stratum Corneum • contains nuclei • Stratum granulosum • doesn’t exist • Stratum Spinosum • expanded • bulbous downward projections • mitosis
Disorders of KeratinizationPsoriasis • Pathology contd. • Papillary Dermis • Papillae thickened • large dilated thin walled blood vessels • Dermis/Epidermis • infiltrated with leukocytes • in Stratum corneum these clump together to form Spongiform pustules (aka Munro microabscesses)
Psoriasis Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)
Psoriasis Koebner Phenomenon Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)
Required Reading • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992) • Psoriasis
Disorders of KeratinisationIcthyoses • A variety of hereditary keratinisation disorders • visible scales on the skin • Forms include • autosomal dominant • x-linked • associated with multisystem changes
Disorders of KeratinisationIcthyoses • May vary from very mild to very severe • The keratinisation process which is changed varies from condition to condition
Icthyosis Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)
Required Reading • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. on Keratinization and Blistering Syndromes
Dermatitis/eczema • dermatitis = eczema • non-infective inflammation of the skin • Greek for ‘to boil over’ • reaction to various stimuli • some known, some unknown
DermatitisClassification • current classification unsatisfactory/inconsistent • distinctions are often difficult to determine • endogenous (internal factors) • exogenous (external factors) • acute • chronic
DermatitisAcute • acute eczema leads to epidermal oedema (spongiosis), with separation of keratinocytes • leads to epidermal vesicles • dermal vessels become dilated • inflammatory cells invade the dermis and epidermis
DermatitisChronic • chronic eczema leads to a thickening of the stratum spinosum (acanthosis) & stratum corneum (hyperkeratosis) • also get retention of nuclei by some corneocytes • rete ridges are lengthened • dermal vessels are dilated • inflammatory mononuclear cells infiltrate the skin
Dermatitis Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)
DermatitisTypes of dermatitis/eczema • contact dermatitis/eczema • contact with an irritant • atopic dermatitis/eczema • associated with a history of asthma, allergic rhinitis, conjunctivitis • seborrhoeic dermatitis/eczema • commonly affects the scalp and face
DermatitisTypes of dermatitis/eczema cont. • discoid (nummular) dermatitis/eczema • often presents as coin-shaped lesions on the limbs of middle aged or older people • venous stasis dermatitis/eczema • associated with venous disease • commonly involves the medial aspect of the ankle
Required Reading • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston., D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. • Chapters on Eczema
Bullous Disorders • blistering (bullous) disorders are often seen with skin disease • found with common skin conditions like acute contact dermatitis • Etiology-autoimmune mechanisms, inheredited errors in metabolism and mechanical trauma
Pemphigus Pemphigoid Epidermolysis bullosa dermatitis herpetiformis linear lgA disease Fungi Friction Systemic lupus erythematosis (SLE) Erythema multiforme Stevens-Johnson syndrome Bullous impetigo Bullous diabeticorum Types of Bullous Disorders
Bullous disorders • Blisters are classified according to their position in the epidermis. • SubCorneal :Stratum Corneum • Intraepidermal: Lower levels of the epidermis • Sub Epidermal: At the dermo-epidermal junction
Friction Blisters • direct mechanical trauma • Treatment: avoidance-look at footwear, protective taping and padding, 2 pairs of socks, lubrication. • sock design and reduction of blistering • Ref.-Herring and Ritchie in JAPMA 1990 and 1993.
Required Reading • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. • Read Chapter on Blistering Disorders and chapter on Keratinization and Blistering Syndromes
inappropriate or exaggerated response to the degree that tissue damage occurs. 4 Types Type l -immediate Type ll -antibody dependant cytotoxicity Type lll-immune complex disease Type lV-cell mediated or delayed Hypersensitivity Reactions and the Skin