KAP ANNUAL SCIENTIFIC CONFERENCE · Ulc=Mucocutaneous ulcers OI= Opportunistic infections...
Transcript of KAP ANNUAL SCIENTIFIC CONFERENCE · Ulc=Mucocutaneous ulcers OI= Opportunistic infections...
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KAP ANNUAL SCIENTIFIC CONFERENCE
Grand Regency Hotel 18. March 2008
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MULTIDISCIPLINARY APPROACHTO MANAGEMENT OF THE PATIENTWITH SYSTEMIC DISEASE AND SKINLESIONS.
Dr. T.M.Munyao.
Senior LecturerDepartment of Clinical Medicine andTherapeutics
University of Nairobi
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Outline
• Multidisciplinary approach: InterpretationBasis
• Skin Syndromes and signs of Systemic disease• Performance of Multidisciplinary approach • Failure of Multidisciplinary approach:• Conclusions
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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
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SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE
1. DERMATOLOGICAL EMERGENCIES.
2. OTHER SKIN SIGNS.
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SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE
1. Dermatological emergencies.Skin failure syndrome• Steven Johnson Syndrome • Toxic Epidermal Necrolysis • Exfoliative erythroderma.• Vesiculobullous disorders >30% BSAB. Shock• Generalised UrticariaC. Upper Airway Obstruction• Orofacial angioneurotic oedema
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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.
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Toxic Epidermal NecrolysisDefinition:>30% epidermal
detachment• Spontaneous or evolving from
SJS.Aetiology ^ Risk SLE, HIV/AIDS:drugs, Infections: Viral, bacterial, fungal NeoplasmsCollagen vascular disorders
Systemic effects:• Functional skin failure
syndrome.Fluid and electrolyte imbalance,Infections,Tracheobronchitis,Meningism , synechiae, phimosisPrognosis: worst: Mortality >30%
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Steven Johnson SyndromeDefinition: Prodrome, Mucocutaneous:Mucositis >2 sites, Target erythema, macules,vesicles, bullae, < 10% body surface areaepidermal detachment, systemic:Nephritis, Tracheobronchitis, Meningism Aetiologydrugs, Infections: Viral, bacterial, fungal NeoplasmsEndocrine, Collagen vascular disorders
Systemic effects:As above and Fluid and electrolyte imbalance,Infections, synechiae,Prognosis: Mortality >10%
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Exfoliative erythrodermaDefinition: Generalised erythema and
ScaleAetiology:• 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%
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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.
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Urticaria & Angioneurotic oedemaDefinition: Diffuse Pruritic dermal or subcutaneous oedema due vasoactivemast cell or complement cascadeproducts.ClassificationHereditary, Neoplasms, Bee stingsSensitisation, Parasitoses, Drugreactions, Atopy, PregnancyEffectsUrticaria : risk of shock.
Angioneurotic oedemaGrotesque facies, risk of airway obstruction
Day 2
Day 6
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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
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PruritusDefinition: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
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Signs of Pruritus: Excoriation Marks
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Signs of Pruritus: Polished Nails
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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 diseasesb. 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
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Albinism with Basal Cell Ca.
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SLE With vitiligo
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Addison's disease
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Erythema1. Vascular Nevoid disorders: Sturge weber, Kassabach Meritt, Klippel
Trenaunay Syndrome
2. Purpura and ecchymoses- Coagulopathy, Scurvy: Ascorbic acid,
3. VasculitidesPolyarteritis nodosa, Churg strauss, Giant cell arteritis,Hypersensitivity, Kawasaki disease, Henoch Schonlein,Goodpastures, Infections, Wegeners granulomatosis, Diffuse
4. Flush –Carcinoid
5. Figurate ErythemasErythema multiforme- Viral Infections, Adverse Cutaneous drug reactions Erythema marginatum- Rheumatic feverErythema gyratum repens- Internal malignancyErythema Chronicum migrans- Lyme disease
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Sturge Weber Syndrome
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Purpura
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Photosensitivity• Ultraviolet light induced inflammation.• Erythema on exposed areas.• Diverse aetiologies:
Genodermatoses: Porphyrias, Xeroderma pigmentosumNutritional deficiency: PellagraCollagen vascular diseases (CVD): Systemic Lupus Erythematosus, Dermatomyositis, Overlap syndromes…
Neoplasms: Paraneoplastic CVD.Adverse cutaneous drug reactions: Tetracyclines,
Phenothiazines, Amiodarone
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Xeroderma Pigmentosum
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Dermatomyositis with Heliotrope Erythema
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Discoid Lupus Erythematosus
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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 PathogensViruses: Epidermodysplasia verruciformis, Verr. plana Herpesviruses HSV, VZV, Molluscum contagiosumBacteria: Pyogenic, Bacillary angiomatosis, septicaemia.Fungi: Yeasts Candidiasis, Cryptococcosis,
Histoplasmosis, Dermatophytes• Frequently atypical, overwhelming and recalcitrant.
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Herpes Zoster
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Verruca Vulgaris
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Opportunistic Neoplasms
• Immunocompromised host.
• carcinogenic factors: Viruses, Impaired immunesurveillance, genetic lesions.
• Kaposis sarcoma, carcinoma of cervix, B Cell lymphoma, Squamous cell carcinoma.
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Kaposis Sarcoma
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Immunosuppression syndrome Squamous cell carcinoma.
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SCLE Squamous cell carcinoma.
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Papulosquamous disordersPsoriasis• 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
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Psoriasis vulgaris in Type I D.M
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KeratodermaDefinition: Diffuse hyperkeratosis most marked on
physiologically drier parts: limbs
Diverse aetiologies:Genodermatoses: Anhidrotic ectodermal dysplasia, IcthyosesDiabetes Mellitus: Acanthosis NigricansChronic inflammation: Acquired icthyosis: ISSyndrome, leprosy Reiter's syndrome: Keratoderma blenorhagicumNeoplasms: Howell Evans Syndrome Carcinoma of oesophagus, Acanthosis Nigricans
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Acquired Icthyosis
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Mucocutaneous ulcers• Cutaneous tissue defect involving epidermis
and at least part of dermis.
Diverse aetiologies:Hemoglobinopathies: SCD, Thalassaemia, , Infections: GUD, Leprosy, Anthrax, NeuropathiesPeripheral vascular disease: GangrenePyoderma gangrenosumNeoplasmsMetabolic: Diabetes mellitus,
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Pyoderma gangrenosumPyoderma gangrenosum
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Squamous cell carcinoma.
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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 NeoplasmsHistiocytoses, Mastocytoses, Pseudolymphomas, CTCLymphoma, Kaposis sarcoma, Leukemia cutis
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Neurofibromatosis
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Others:
• Eczema: Atopy, Anasarca, Varicose veins, Wiskott Aldrich, PKU, Acrodermatitis enteropathica and acquired Zinc deficiency
• Alopecia: SLE, Malnutrition, Follicular mucinosisProgeria, ADEnteropathica, Telogen effluvium
• Hirsutism: Adrenogenital syndromes• Cutanous sclerosis: systemic sclerosis
Overlap syndromes• Endocrinopathic Acne: Cushings, Virilizing
syndromes.
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SKIN SYNDROMES/SIGNS OF INTERNAL DISEASE
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
SKIN SYNDROMES/ SIGNS
DISEASES 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. ☻ ☻ ☻
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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.
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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 outcomesThreat to continued tenure to professional clout
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ConclusionsFor holistic diagnosis and management of the patient with systemic disease and skin lesions:
MULTIDISIPLINARY APPROACH must including a Dermatologist.
THE ONLY PROFESONAL APPROACH
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THE END
THANK YOU