KAP ANNUAL SCIENTIFIC CONFERENCE. Grand Regency Hotel 18. March 2008
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1 KAP ANNUAL SCIENTIFIC CONFERENCE Grand Regency Hotel 18. March 2008
2 MULTIDISCIPLINARY APPROACH TO MANAGEMENT OF THE PATIENT WITH SYSTEMIC DISEASE AND SKIN LESIONS. Dr. T.M.Munyao. Senior Lecturer Department of Clinical Medicine and Therapeutics University of Nairobi
3 Outline Multidisciplinary approach: Interpretation Basis Skin Syndromes and signs of Systemic disease Performance of Multidisciplinary approach Failure of Multidisciplinary approach: Conclusions
4 Multidisciplinary approach: Interpretation: Multidisciplinary: Internists BASIS: Systemic diseases with skin lesions. Mechanisms of Multisystemic involvement: unitary primary aetiopathogenesis. eg SLE Activation of effector pathways. eg Urticaria Compromised effector pathways. eg ISSyndrome Metabolites with similar physical properties. eg Jaundice, Carotenaemia, Xanthomatoses
5 SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE 1. DERMATOLOGICAL EMERGENCIES. 2. OTHER SKIN SIGNS.
6 SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE 1. Dermatological emergencies. Skin failure syndrome Steven Johnson Syndrome Toxic Epidermal Necrolysis Exfoliative erythroderma. Vesiculobullous disorders >30% BSA B. Shock Generalised Urticaria C. Upper Airway Obstruction Orofacial angioneurotic oedema
7 SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE 1. Dermatological emergencies. Associated with adverse outcomes due to complications culminating in multiorgan failure Prognosis depended on syndrome specific composite criteria Best Prognosis with prompt holistic diagnosis and Multidisciplinary approach to management.
8 Toxic Epidermal Necrolysis Definition:>30% epidermal detachment Spontaneous or evolving from SJS. Aetiology ^ Risk SLE, HIV/AIDS: drugs, Infections: Viral, bacterial, fungal Neoplasms Collagen vascular disorders Systemic effects: Functional skin failure syndrome. Fluid and electrolyte imbalance, Infections,Tracheobronchitis, Meningism, synechiae, phimosis Prognosis: worst: Mortality >30%
9 Steven Johnson Syndrome Definition: Prodrome, Mucocutaneous: Mucositis >2 sites, Target erythema, macules, vesicles, bullae, < 10% body surface area epidermal detachment, systemic: Nephritis, Tracheobronchitis, Meningism Aetiology drugs, Infections: Viral, bacterial, fungal Neoplasms Endocrine, Collagen vascular disorders Systemic effects: As above and Fluid and electrolyte imbalance, Infections, synechiae, Prognosis: Mortality >10%
10 Exfoliative erythroderma Definition: Generalised erythema and Scale Aetiology: Dermatoses: Eczema, Psoriasis Systemic diseases: HIV/AIDS, Lymphoreticular and other neoplasms Adverse drug reactions. Effects: Hypothermia, infections, Hypoproteinaemia, Renal and Cardiac failure Prognosis: Mortality: Global 30-70%, KNH 10%
11 Vesiculobullous disorders >30% BSA Commonly due to Immunobullous disease: Pemphigus, Pemphigoid Non immunobullous causes: Infections. Ssssyndome, Varicella, Herpes zoster, Disseminated Herpes simplex Metabolic- Diabetes mellitus, Porphyria Paraneoplastic Pemphigus Functional skin failure syndrome.
12 Urticaria & Angioneurotic oedema Definition: Diffuse Pruritic dermal or subcutaneous oedema due vasoactive mast cell or complement cascade products. Classification Hereditary, Neoplasms, Bee stings Sensitisation, Parasitoses, Drug reactions, Atopy, Pregnancy Effects Urticaria : risk of shock. Angioneurotic oedema Grotesque facies, risk of airway obstruction Day 2 Day 6
13 2. Other skin signs of Systemic disease. Dermatological emergencies. a. Skin failure syndrome Steven Johnson Syndrome Toxic Epidermal Necrolysis Bullous disease >30% body surface area Exfoliative erythroderma. b. Shock Generalised Urticaria c. Upper Airway Obstruction Urticaria/angioneurotic oedema Pruritus Non erythematous Pigmentation. Erythema/purpura, Vasculitides/Figurate erythema Photosensitivity Opportunistic infections/neoplasms Papulosquamous disorders Keratoderma. Mucocutaneous ulcers Papules, Nodules, Plaques and Patches
14 Definition: Pruritus Sensory modality which provokes scratching. Clinical signs: Excoriation marks and polished nails Associated causative factors Associated Systemic diseases: Obstructive jaundice, Urticaria/Angioneurotic oedema, Pregnancy, Drugs, Neurosis, Paraneoplastic: Lymphoma, Polycythaemia rubra vera
15 Signs of Pruritus: Excoriation Marks
16 Signs of Pruritus: Polished Nails
17 Non Erythematous Pigmentation disorders. 1. Pallor: Anaemia, shock 2. Melanin: a. Amelanoses: Albinism : Global amelanosis due to Tyrosinase abnormalities Associated with eye and neurological abnormalities. Vitiligo: Macules, Patches: Associated with diffuse collagen vascular and other autoimmune diseases b. Hypermelanoses Global: Addison s disease, Focal: Macules, Patches: Melasma, Café au lait, 3. Yellow; Jaundice: Hemolysis, Biliary obstruction, Chronic liver disease, Carotenaemia, Xanthomatoses, Yellow Nail syndrome 4. Blue/Blue Gray: Cyanosis, Ochronosis (Coeruloderma) Homogentisic acid polymer, Slate grey: Haemochromatosis 5. Composite: Raynauds phenomenon
18 Albinism with Basal Cell Ca.
19 SLE With vitiligo
20 Addison's disease
21 Erythema 1. Vascular Nevoid disorders: Sturge weber, Kassabach Meritt, Klippel Trenaunay Syndrome 2. Purpura and ecchymoses- Coagulopathy, Scurvy: Ascorbic acid, 3. Vasculitides Polyarteritis nodosa, Churg strauss, Giant cell arteritis, Hypersensitivity, Kawasaki disease, Henoch Schonlein, Goodpastures, Infections, Wegeners granulomatosis, Diffuse 4. Flush Carcinoid 5. Figurate Erythemas Erythema multiforme- Viral Infections, Adverse Cutaneous drug reactions Erythema marginatum- Rheumatic fever Erythema gyratum repens- Internal malignancy Erythema Chronicum migrans- Lyme disease
22 Sturge Weber Syndrome
23 Purpura
24 Photosensitivity Ultraviolet light induced inflammation. Erythema on exposed areas. Diverse aetiologies: Genodermatoses: Porphyrias, Xeroderma pigmentosum Nutritional deficiency: Pellagra Collagen vascular diseases (CVD): Systemic Lupus Erythematosus, Dermatomyositis, Overlap syndromes Neoplasms: Paraneoplastic CVD. Adverse cutaneous drug reactions: Tetracyclines, Phenothiazines, Amiodarone
25 Xeroderma Pigmentosum
26 Dermatomyositis with Heliotrope Erythema
27 Discoid Lupus Erythematosus
28 Opportunistic Infections Immunocompromised host. Diverse causes: HIV/AIDS, Iatrogenic, Congenital, Chediak Higashi Syndr., Wiskott Aldrich Syndr., Chronic Gran. Dis., Diabetes Mellitus, Malnutrition, Oncology, Debilitation,.. Diverse Pathogens Viruses: Epidermodysplasia verruciformis, Verr. plana Herpesviruses HSV, VZV, Molluscum contagiosum Bacteria: Pyogenic, Bacillary angiomatosis, septicaemia. Fungi: Yeasts Candidiasis, Cryptococcosis, Histoplasmosis, Dermatophytes Frequently atypical, overwhelming and recalcitrant.
29 Herpes Zoster
30 Verruca Vulgaris
31
32
33 Opportunistic Neoplasms Immunocompromised host. carcinogenic factors: Viruses, Impaired immunesurveillance, genetic lesions. Kaposis sarcoma, carcinoma of cervix, B Cell lymphoma, Squamous cell carcinoma.
34 Kaposis Sarcoma
35 Immunosuppression syndrome Squamous cell carcinoma.
36 SCLE Squamous cell carcinoma.
37 Papulosquamous disorders Psoriasis Overwhelming evidence for immunopathogenesis. Vulgaris most frequent variant. Erythrodermic and Pustular variants rare but serious. Comorbids include: Arthropathy, Type I Diabetes mellitus, Hypothyroidism, Asthma, polycystic ovarian syndrome Lichen planus Comorbid with autoimmune disorders Lichenoid drug eruptions with 4 amminoquinolines, and chrysotherapy. Chronic GVH disease is lichenoid
38 Psoriasis vulgaris in Type I D.M
39 Keratoderma Definition: Diffuse hyperkeratosis most marked on physiologically drier parts: limbs Diverse aetiologies: Genodermatoses: Anhidrotic ectodermal dysplasia, Icthyoses Diabetes Mellitus: Acanthosis Nigricans Chronic inflammation: Acquired icthyosis: ISSyndrome, leprosy Reiter's syndrome: Keratoderma blenorhagicum Neoplasms: Howell Evans Syndrome Carcinoma of oesophagus, Acanthosis Nigricans
40 Acquired Icthyosis
41 Mucocutaneous ulcers Cutaneous tissue defect involving epidermis and at least part of dermis. Diverse aetiologies: Hemoglobinopathies: SCD, Thalassaemia,, Infections: GUD, Leprosy, Anthrax, Neuropathies Peripheral vascular disease: Gangrene Pyoderma gangrenosum Neoplasms Metabolic: Diabetes mellitus,
42 Pyoderma gangrenosum Pyoderma gangrenosum
43 Squamous cell carcinoma.
44 Papules, Nodules, Plaques and Patches Pregnancy: PUPP Metabolic disorders; Metabolic storage diseases and inborn errors of Metabolism: Glycogen, mucopolysacchridoses, Lipids, Amyloid, Lipoid proteinosis. Genodermatoses: Neurofibromatosis, Tuberose sclerosis Dermal cellular hyperproliferations and Neoplasms Histiocytoses, Mastocytoses, Pseudolymphomas, CTCLymphoma, Kaposis sarcoma, Leukemia cutis
45 Neurofibromatosis
46 Others: Eczema: Atopy, Anasarca, Varicose veins, Wiskott Aldrich, PKU, Acrodermatitis enteropathica and acquired Zinc deficiency Alopecia: SLE, Malnutrition, Follicular mucinosis Progeria, ADEnteropathica, Telogen effluvium Hirsutism: Adrenogenital syndromes Cutanous sclerosis: systemic sclerosis Overlap syndromes Endocrinopathic Acne: Cushings, Virilizing syndromes.
47 SKIN SYNDROMES/SIGNS OF INTERNAL DISEASE DISEASES SKIN SYNDROMES/ SIGNS TEN SJS Exf. VB U/A Pru Pig. Ery. F.ery Pho Ulc OI Vas Pnp Infections Drugs Neoplasms Coll.vas.dis. Parasitoses Pregnancy Endocrine Haematol. Neurocut. KEY TEN= Toxic Epidermal Necrolysis SJS=Steven Johnson Syndrome Pru= Pruritus Pig= Pigmentation Ery= Erythema VB= Vesiculobullous Coll.vas.dis. = Collagen vascular diseases. F.ery=Figurate erythema Pho=Photosensitivity Ulc=Mucocutaneous ulcers OI= Opportunistic infections Vas=Vasculitis Pnp=Papules, nodules, plaques Neurocut. = Neurocutaneous Exf. =Exfoliative erythroderma U/A= Urticaria Angioneurotic oedema
48 Performance of Multidisciplinary approach: Specialists most current in knowledge and professional experience and competence. Multidisciplinary approach: for holistic diagnosis and plan of management. Provide best quality of care and management targets.
49 Failure of Multidisciplinary approach Patient denied access to most qualified Specialists. Diagnosis untenable and plan of management impossible. Natural course of disease. Undiagnosed: Risk of adverse outcomes Threat to continued tenure to professional clout
50 Conclusions For holistic diagnosis and management of the patient with systemic disease and skin lesions: MULTIDISIPLINARY APPROACH must including a Dermatologist. THE ONLY PROFESONAL APPROACH
51 THE END THANK YOU
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