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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


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Disorders of Keratinisation

  • Overview

  • Psoriasis

  • Icthyosis


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Objectives

  • Disorders of Keratinisation


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Disorders of Keratinization

Keratinization

Terminal differentiation of epithelia

  • epithelial proteins (Keratin)

  • Glycoproteins (Cell Envelope)

  • intercellular lipids


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Disorders of Keratinization

  • Cause changes in the skin

    • Dry, Scaly, Thickened, Flaky

    • Blistering

  • Cause changes in Mucous membranes, Nails and Hair


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Disorders of Keratinization

  • Change in Type of Keratin Made




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Disorders of Keratinization

  • Psoriasis

  • Icthyosis


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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


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Disorders of KeratinizationPsoriasis

  • Characterised by hyperproliferation of skin and inflammation


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Disorders of KeratinizationPsoriasis

  • Etiology

    • Inherited

      • Abnormality of Ca++ metabolism

      • Genetic Predisposition

        • HLA Cw6

        • HLA DR7

        • HLA B27 (Pustular)


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Disorders of KeratinizationPsoriasis

  • Etiology

    • Environmental Factors

      • Stress, Smoking and Alcohol

      • Systemic Drugs

      • Infection

    • Immunological Factors


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Psoriasis

Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)


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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


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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


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Disorders of KeratinizationPsoriasis

  • Pathology

    • Stratum Corneum

      • contains nuclei

    • Stratum granulosum

      • doesn’t exist

    • Stratum Spinosum

      • expanded

      • bulbous downward projections

      • mitosis


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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)


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Psoriasis

Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)


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Psoriasis

Koebner Phenomenon

Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)


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Required Reading

  • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)

  • Psoriasis


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Disorders of KeratinisationIcthyoses

  • A variety of hereditary keratinisation disorders

  • visible scales on the skin

  • Forms include

    • autosomal dominant

    • x-linked

    • associated with multisystem changes


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Disorders of KeratinisationIcthyoses

  • May vary from very mild to very severe

  • The keratinisation process which is changed varies from condition to condition


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Icthyosis

Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)


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Required Reading

  • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. on Keratinization and Blistering Syndromes


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Dermatitis/eczema

  • dermatitis = eczema

  • non-infective inflammation of the skin

  • Greek for ‘to boil over’

  • reaction to various stimuli

    • some known, some unknown


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DermatitisClassification

  • current classification unsatisfactory/inconsistent

  • distinctions are often difficult to determine

  • endogenous (internal factors)

  • exogenous (external factors)

  • acute

  • chronic


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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


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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


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Dermatitis

Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston. (1992)


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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


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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


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Required Reading

  • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston., D.J. (1992) Dermatology. Edinburgh: Churchill Livingston.

  • Chapters on Eczema


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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


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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


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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



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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.


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Required Reading

  • Gawkrodger, D.J. (1992) Dermatology. Edinburgh: Churchill Livingston.

  • Read Chapter on Blistering Disorders and chapter on Keratinization and Blistering Syndromes


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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