PSORIASIS. -Multi factorial. -Papulosquamous disorder. -Genetically determined (few) -Chronic Scaly lesions. -Seasonal variations



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Transcription:

PSORIASIS -Multi factorial -Papulosquamous disorder -Genetically determined (few) -Chronic Scaly lesions -Seasonal variations -Recurrences & remissions

Etiology & Pathogenesis T-cell mediated autoimmune disease -Early onset Hereditary HLACW6 -Late Onset Non inheritable

Genetic Predisposition - Multifactorial - Familial aggregation - HLA B13, HLA B17, HLACW6

PREDISPOSITION -Familial - Risk 3 times one parent -60% children both parent -Genetic -Autosomal dominant -Twin heritability - 90% -HLA B 13 B 17 B 27

PROVOKING/PRECIPITATING FACTORS -Predisposed individual -TRAUMA (Isomorphic/Koebner s phenomena) -INFECTION ( Streptococcal Guttate Psoriasis) -PREGNANCY( Pustular Psoriasis) -CLIMATE : Summer,Winter -HYPOCALCEMIA

Drugs -Anti malarial -B-Blockers -Lithium -High dose steroid (sudden withdrawal) Psychogenic factors Dialysis

Mechanism of Proliferation Cell cycle time Basal epidermis cycling & resting cells Recruitment of cycling cells Growth fraction Accelerated epidermopoiesis

HISTOPATHOGENESIS Accelerated epidermopoiesis- Main feature Parakeratosis- Nucleated cells in s.corneum Absence of granular layer Presence of PMNL Intradermal abscess of PMNL - Spongiform pustules of Kogoj - Munro microabscesses

Mechanism Immunological disease -Cyclic AMP,Cyclic GMP - Arachidonic acid & by products like PG (HETE), Leukotrienes - Polyamines : Putresine & spermidine - Proteinase activity - Interleukins - Activity of PMNL, Langerhans cells, monocytes

HPE : Variable Acanthosis Parakeratosis, absence of granular layer Elongation of rete ridges PMNL infiltrate in dermis Munro s micro abscess (epidermis)

Clinical Features Both sexes equally affected Females earlier Age of onset 16-22 years 57-60 years

PSORIASIS VULGARIS -Both sexes, -All ages (20-40 years) Clinically Extensors, palms, soles, scalp Well defined lesions Scaly plaques, papules

Characteristic sign Easily removable silvery scales GRATTAGE SIGN AUSPITZ SIGN Halo of Woronoff-clear peripheral zone

Koebner phenomena present

Modification by site Scalp -Asbestos like scaling -Pityriasis amiantacea

Flexural - No scaling, - Glazed hue

Flexural Psoriasis

Napkin Psoriasis Penis No scaling Linear/Zonal psoriasis

Palmoplantar Psoriasis Palms/ soles

NAIL CHANGES (25-50%) Distal onycholysis Subungual hyperkeratosis Oil drop sign Pitting Destruction/Discoloration

NAIL CHANGES

Nail Matrix - Pitting - Ridges & grooves Nail Bed - Subungual hyperkeratosis - Onycholysis

Nail Changes

Clinical Variants Psoriasis vulgaris

Elephantine Psoriasis

Rupioid Psoriasis Ostraceous Psoriasis Atypical psoriasis Guttate Psoriasis

Guttate Psoriasis Streptococcal throat infections Child Adolescent Monomorphic Respond to antibiotics May lead to Psoriasis vulgaris in predisposed

Guttate Psoriasis

Erythrodermic Psoriasis -Some ppt. factor -Disease characteristics may be lost -Febrile -Severe itching

Metabolic complications - Hypo/Hyperthermia - High output cardiac failure - Hypoalbuminaemia - Malabsorption

Pustular Psoriasis Localized -Chronic -Palmoplantar

Pustular Psoriasis Generalized -Von Zumbusch -Acute

Pustular psoriasis Withdrawal of steroid Erythrodermic psoriasis

Impetigo Herpetiformis -Pregnancy -Onset in last trimester -Starts in flexures -Severe constitutional disturbance -Risk of placental insufficiency -Recurs

Psoriatic arthritis -Seronegative -HLA B27

Psoriatic arthropathy -Distal symmetrical I.P. -Oligo- mono arthritis -Seronegative Rh. Arthritis like arthritis -Psoriatic arthritis multilans -Ankylosing spondylitis like

X-ray Findings - Local demineralization - Pencil in cup deformity

Course & Prognosis - Chronic - Intractable - Relapse is the rule

TREATMENT a)topical -5% Crude coal tar(goekerman s regimen) Dithranol (anthranilin) 1-2% (short contact therapy) Combined coal tar + Dithranol (Ingram regimen) Topical steroids (mild) UV-B Therapy

Topical treatment Contd. Liquor Picis carbonis Keratolytics Emollients Topical PUVA Topical Methotrexate Calcipotriol Vit. D analogue

b) Systemic Therapy Methotrexate (0.3-0.5mg/kg/week) PUVA Psoralens + UVA Retinoids etretinate/ acitretin 0.5mg/kg/day Cyclosporine Corticosteroids

Thank You