Photosensitive disorders and Urticaria Sunlight and Skin Major source of UV and visible radiation interacting with human skin. UV radiation : UVC: 200-290 nm UVB: 290-320 nm UVA: 320-400 nm Visible radiation: 400-760 nm Infrared radiation:760 -100,000 nm Normal effects of sun exposure on skin: Sunburn, Tanning, Photoaging, Vitamin D synthesis. Skin Phototypes(Fitz Patrick Classification) I - always burns, never tans II - always burns, minimally tans III - burns moderately, gradually tans IV - burns minimal, always tans V - burns rarely , severely tans VI - deeply pigmented. Indian skin phototypes: Type IV-VI Classification of Photosensitive disorders Genodermatoses ◦ Xeroderma pigmentosum, Bloom’s syndrome, Cockayne syndrome Idiopathic ◦ Polymorphic light eruptions, Actinic prurigo, solar urticaria Phototoxic and photoallergic reactions ◦ Reactions induced by topical/systemic drugs, chemicals or contactants Contd… Classification of Photosensitive disorders Metabolic ◦ Porphyria, Pellagra Tumours ◦ Basal cell ca, Squamous cell ca, Melanoma, Actinic keratoses. Diseases aggravated by sun exposure ◦ Lupus Erythematosus, Rosacea, Dermatomyositis, Acne, Psoriasis, Pemphigus, Atopic dermatitis, Airborne Contact dermatitis Polymorphous light eruption (PMLE) Most common photodermatoses Idiopathic, probably immunological Younger age, Females Recurrent pruritic lesions on photo exposed areas within minutes to hours after sun exposure. Clinical patterns: ◦ Macular, papular,papulovesicles, plaque,lichen-nitidus like lesions. Polymorphous light eruption (PMLE) Differential Diagnosis: ◦ Prurigo ◦ Erythema multiforme ◦ Lichen Simplex chronicus ◦ Atopic dermatitis Photocontact dermatitis PHOTOTOXIC Common Non immunological Sunburn Clinically diagnosed PHOTOALLERGIC Less Common TYPE IV Hypersensitivity Eczematous Photo patch test Systemic/ topical drugs, chemicals contactants in combination with UVA spectrum induces phototoxic and photoallergic reactions. Phototoxic reactions Topical: ◦ Perfumes, Dyes, psoralens, tars, plants (lime, celery) Systemic: ◦ Psoralen, Tetracycline, Phenothiazine Photoallergic reactions Topical: ◦ Perfumes (soaps, aftershave) sunscreens (PABA)neomycin, halogenated compounds parthenium (congress grass) Systemic: ◦ NSAIDS, phenothiazine, thiazides. Approach to photosensitive disorder Detailed history : ◦ Age/ Sex ◦ Occupation, Hobbies ◦ Family h/o similar disease ◦ Time interval between exposure & symptoms ◦ Contactant, drug history ◦ Cosmetics, sunscreen usage. Approach to photosensitive disorder Clinical diagnosis : Photoexposed areas: ◦ Face, ‘V’ area& nape of neck, dorsae of hands & feet, extensors of arms & legs (check clothing used) Spared areas: ◦ Under hair fringe, chin, nose, upper eyelids, behind ears. Morphological pattern depends on etiology Investigations Depending on etiology : ◦ Connective Tissue diseases, Metabolic, Genetic AnA, dsDNA, porphyrin levels (blood, urine,stool), Chromosomal analysis, cell mutation studies, DNA repair assesment Biopsy Photo testing Photopatch testing Treatment: Photoprotection Natural: Ozone, Melanin, Keratin. Physical: Clothing, broad rimmed hats, umbrellas. Topical: Sunscreens, UVA, UVB therapy. Systemic: Beta carotene, Chloroquin, Hydroxychloroquin, PUVA Treatment of causative factor Removal of offending drug/chemical/contactant Steroids (topical/systemic) Immunosuppressives (Azathioprine, cyclosporin, Thalidomide) Sunscreens - Topical Physical Reflect, scatter light Zinc oxide Titanium dioxide Ferrous oxide Chemical Absorbs light & prevents penetration through skin Anthranilates, benzophenones, cinnamates, salicylates Sunscreens - Topical Broad spectrum - UVA & UVB protective SPF 15 or higher in Indian skin Proper application & use in all seasons needed. Liberal and uniform application to photo exposed areas, half an hour prior to sun exposure. 1ml – Face – Female 1.5ml – Face – Male Frequent reapplication if involved in vigorous exercise, swimming. Sunscreens - Topical Broad spectrum - UVA & UVB protective SPF 15 or higher in Indian skin Proper application & use in all seasons needed. Liberal and uniform application to photo exposed areas, half an hour prior to sun exposure. 1ml – Face – Female 1.5ml – Face – Male Frequent reapplication if involved in vigorous exercise, swimming. Urticaria It is a reaction pattern consisting of erythematous, transient, itchy swellings (wheals) on the skin Urticaria is derived from the word “Urtica” (in Latin = Stinging Nettle) Pathogenesis Enhanced vasodilatation + Vaso permeability Plasma leakage Wheals Histamine stimulates nerve endings Pruritus Neuro-peptide release Skin response (flare) Response to histamine release from cutaneous mast cells Often seen with Angioedema Causes Exogenous Endogenous Physical Emotional stress Idiopathic Contd… Causes EXOGENOUS ◦ Inhalants: pollens, house dust, fungi, dander. ◦ Ingestants: Foods: fish, egg, brinjal, food additives, dyes, preservatives. ◦ Drugs: penicillin, NSAIDs, sulfonamides. ◦ Contactants: beestings, insect bites, plants. Causes ENDOGENOUS ◦ Infections: Gastrointestinal, respiratory, urinary tract infections Bacterial, protozoal, helminthic, viral (CMV, EBV, HSV) ◦ Systemic diseases: Hashimoto's Thyroiditis Systemic Lupus Erythematosus Chronic active hepatitis Malignancies Causes PHYSICAL URTICARIA Cold Urticaria ◦ Affects hands, ear, nose and lateral thighs Cholinergic Urticaria ◦ Fever ◦ Hot baths ◦ Exercise-Induced Urticaria Solar Urticaria (Sun induced) Pressure ◦ Tight clothing ◦ Soles of foot and other weight bearing points ◦ Dermographism Types of urticaria by duration: Acute Urticaria (present hours to weeks) ◦ Idiopathic in 75% of cases Chronic Urticaria (persistent beyond 6 weeks) ◦ Idiopathic in 95% of cases ◦ May be related to autoantibody to IgE Clinical features: Symptoms ◦ Pruritus Signs ◦ Characteristics Hives or wheals Spreads with scratching and coalesce to form large patch Course of Lesions ◦ Lesions last 90 minutes to 24 hours Dermographism Dermographism is a condition where linear wheals are elicited by stroking or scratching the skin These wheals subside within 30 minutes Some patients have chronic dermographism which may persist throughout life Angioedema Non-pitting subcutaneous swelling ◦ Well demarcated ◦ Distribution Face Hands Buttocks Genitalia Abdomen Laryngeal angioedema (Anaphylaxis) Anaphylaxis is a medical emergency Workup in a patient of Urticaria Careful History Travel and work history Ingestion of foods, medications, herbals, vitamins Recent infection Negative history makes finding cause more difficult Known allergies Family History of allergy or Thyroid disease Investigations Consider brief panel depending on history i. Hb, CBC ii. Urine analysis iii. Stool analysis iv. ESR v. Liver Function Tests vi. TSH vii. ANA Skin biopsy if lesion present >24 hrs, consider urticarial vasculitis Autologous Serum test Treatment Management : General ◦ Rule out Anaphylaxis ◦ Discontinue offending drugs, food, or behavior ◦ Offer Reassurance Discuss idiopathic nature of chronic urticaria Unlikely to identify a specific cause Contd… Treatment Step 1: Non-Sedating Antihistamines (e.g. Fexofenadine) ◦ Less effective antipruritic as compared to sedating Antihistamine ◦ Consider for daytime urticaria symptom control Step 2: Sedating Antihistamines (e.g. Hydroxyzine) ◦ Consider for night time and refractory to step 1 ◦ Beware of sedation and risk of fall in older patients Step 3: Add H2 Receptor Antagonist (e.g. Ranitidine) Contd… Treatment Step 4: Other anti-histaminics ◦ Doxepin ◦ Cyproheptadine Step 5: Leukotriene modifier ◦ Montelukast ◦ Zafirlukast Step 6: Systemic Corticosteroids ◦ Prednisone 20-40 mg PO qd ◦ Caution : Process may flare when steroids are weaned off Thank you