KAP ANNUAL SCIENTIFIC CONFERENCE

Grand Regency Hotel 18. March 2008

MULTIDISCIPLINARY APPROACHTO MANAGEMENT OF THE PATIENTWITH SYSTEMIC DISEASE AND SKINLESIONS.

Dr. T.M.Munyao.

Senior LecturerDepartment of Clinical Medicine andTherapeutics

University of Nairobi

Outline

• Multidisciplinary approach: InterpretationBasis

• Skin Syndromes and signs of Systemic disease• Performance of Multidisciplinary approach • Failure of Multidisciplinary approach:• Conclusions

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

SKIN SYNDROMES AND SIGNS OF SYSTEMIC DISEASE

1. DERMATOLOGICAL EMERGENCIES.

2. OTHER SKIN SIGNS.

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

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.

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%

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%

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%

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.

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

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

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

Signs of Pruritus: Excoriation Marks

Signs of Pruritus: Polished Nails

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

Albinism with Basal Cell Ca.

SLE With vitiligo

Addison's disease

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

Sturge Weber Syndrome

Purpura

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

Xeroderma Pigmentosum

Dermatomyositis with Heliotrope Erythema

Discoid Lupus Erythematosus

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.

Herpes Zoster

Verruca Vulgaris

Opportunistic Neoplasms

• Immunocompromised host.

• carcinogenic factors: Viruses, Impaired immunesurveillance, genetic lesions.

• Kaposis sarcoma, carcinoma of cervix, B Cell lymphoma, Squamous cell carcinoma.

Kaposis Sarcoma

Immunosuppression syndrome Squamous cell carcinoma.

SCLE Squamous cell carcinoma.

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

Psoriasis vulgaris in Type I D.M

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

Acquired Icthyosis

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,

Pyoderma gangrenosumPyoderma gangrenosum

Squamous cell carcinoma.

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

Neurofibromatosis

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.

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. ☻ ☻ ☻

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.

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

ConclusionsFor holistic diagnosis and management of the patient with systemic disease and skin lesions:

MULTIDISIPLINARY APPROACH must including a Dermatologist.

THE ONLY PROFESONAL APPROACH

THE END

THANK YOU