Emergency Dermatology Dr Melissa Barkham Spotlight Seminar 30 th September 2010

Why is this important?  Urgent recognition and treatment of dermatologic emergencies can be life saving and prevent long term morbidity  How do you differentiate rare life threatening conditions from the common skin complaints that make up % of consultations in primary care?

Skin structure and function  Protective barrier (toxins, microbes, u.v. light, physical injury)  Temperature regulation  Fluid homeostasis  Sensation  Immunological function  Synthetic (e.g. Vitamin D)  Psycho-social

Consequences of Skin failure  Similar to patients with extensive burns  Dehydration  Fluid and electrolyte imbalance  Hypo - albuminaemia  Hypotension  Hypothermia  Sepsis  Other organ failure  (e.g. renal, hepatic, CCF)

Emergency Dermatology Overview Skin signs a diagnostic clue (to serious underlying disorder) Severe Infections (e.g. meningococcaemia, necrotising facsiitis, staphylococcal scalded skin) Acute autoimmune disease (e.g. SLE, systemic vasculitis) Paraneoplastic (e.g. dermatomyositis) Skin disease causing risk of vital organ failure / death Severe adverse drug reactions (e.g. toxic epidermal necrolysis) Erythroderma (e.g. due to extensive inflammatory skin disease ) Autoimmune Blistering disorders (e.g. pemphigus vulgaris)

Team approach  GP  Accident and emergency  On call medical (or paediatric) team  Dermatology consultants and specialist nurses  ITU  Histopathology  Microbiology  …..to name but a few

Cutaneous Adverse Drug Reactions  Common - severity variable  Can be life threatening  Potential long term sequelae (e.g. blindness)  Think carefully before you prescribe any medicine!  Yellow card reporting (MHRA)  Over the counter drugs and supplements can be the culprit

Cutaneous Adverse Drug Reactions  History may not be volunteered  Ask about all medications taken in the last 3 months  Prescribed and non prescribed (including household remedies, herbal remedies, vitamins and supplements)  Beware compound preparations (e.g. cold and flu remedies)

Severe Drug reaction - types  Exanthemous (morbilliform)  Stevens - Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)  Drug hypersensitivity syndrome (DHS)  Urticaria +/- angioedema

Drug reaction - warning signs  Facial or mucous membrane involvement  Widespread erythema  Skin pain  Blistering / skin necrosis  Fever  Lymphadenopathy / arthralgia  Features of anaphylaxis  Other organ involvement (e.g. hepatic or renal dysfunction)

Exanthemous drug reaction - features  Commonest type  Onset 5-10 days after new drug  Morbilliform (measles like) maculopapular rash  Usually itchy  Sometimes associated with fever / malaise  Commoner in patients with infectious mononucleosis, leukaemia or HIV  Suspected drug (or drugs) should be discontinued and rash subsides in 1-2 weeks

Exanthemous drug eruption - culprits  Penicillins  Carbamazepine  Allopurinol  Sulphonamides  NSAIDS  Phenytoin  Isoniazid

DHS - clinical features  Morbilliform rash with fever and internal organ involvement  “Toxic erythema”  Mortality - about 10%  Later onset (2-6 weeks) after new drug commenced  Fever, lymphadenopathy  Eosinophilia (DRESS) in some  Hepatic / renal failure  Treatment: withdrawal of offending drug(s) and supportive care

DHS - culprits  Sulphonamides  Dapsone  Anticonvulsants  ACE inhibitors  Beta - blockers  Allopurinol  Minocycline  SSRI

TEN / SJS - clinical features  Rare drug reaction - presents with skin and mucosal loss  Variants of the same condition (differentiated by extent of skin involved - TEN >30%, SJS <10%)  Mortality - 50%  Mucous membrane involvement (eyes, mouth, genitalia) - can scar  Tender, blistering skin and necrotic epidermis – areas of denuded skin  Positive Nikolsky sign (blisters extend with skin pressure)

TEN / SJS - culprits  More than 100 drugs reported including...  Penicillins  Sulfonamides  NSAIDS (including ibuprofen)  Anticonvulsants  Allopurinol  Antiretrovirals .... and even paracetamol  Susceptibility factors - HIV, genetic susceptibility

TEN / SJS - Differential diagnoses  Erythema Multiforme - self limiting reaction triggered by infections e.g. HSV. Typical target lesions especially on acral sites. May involve mucosae.  Staphylococcal scalded skin syndrome (SSSS) - a localised infection with a toxigenic strain of S. Aureus triggers fever, redness of skin and easily ruptured blisters. Flexures often affected and mucosae uninvolved.  Autoimmune blistering disorders

TEN / SJS - investigations  Skin biopsy for histology and direct immunofluorescence (DIF)  H&E sections - basal or full thickness epidermal keratinocyte necrosis, supepidermal blistering (SSSS - the split is higher)  DIF - negative (rules out autoimmune disease)

TEN / SJS - management  Remove all possible culprit drugs  Supportive care in ITU or high dependency setting (skin failure)  Careful fluid and electrolyte balance  Analgesia  Non - adherent dressings / sheets  Ophthalmology input  Prevention and treatment of secondary infections  Consider intravenous immunoglobulin  Future avoidance (including 1st degree relatives)

Drug induced Urticaria  Drug induced urticaria can occur with or without angioedema  Up to 3 weeks after first exposure (or minutes on re-challenge)  Types -  Type 1 hypersensitivity (e.g. penicillin) - can be associated with anaphylaxis  Mast cell degranulation on first exposure (e.g. NSAIDS, opiates)  Angioedema without urticaria (e.g. ACE inhibitors)

Drug Induced Urticaria - culprits  NSAIDS  Penicillins  Cephalosporins  Sulphonamides  ACE inhibitors  Calcium channel inhibitors  Vaccinations

What is Erythroderma?  Intense and widespread reddening of the skin (more difficult to detect in asian / black skin)  > 90% Body Surface area involement  Often associated with exfoliation (exoliative dermatitis / exfoliative erythroderma)  Often results from exacerbation of a pre-existing skin disorder

Causes of Erythroderma  Psoriasis  Dermatitis  Cutaneous T- Cell lymphoma  Drugs (red man syndrome)  Idiopathic  Paraneoplastic

Erythroderma - management  Identify underlying cause (biopsy)  Consider hospital admission  Supportive care (e.g. keep warm, regular emollients, fluid balance, high protein diet)  Treat underlying disease (e.g. severe psoriasis - methotrexate or other systemics, dermatitis - topical or oral corticosteroids)  Avoid oral corticosteroids in severe psoriasis

Generalised Pustular Psoriasis  Rare form of psoriasis (patient presents with widespread sterile pustules on a background of red and tender skin)  Many have a background of chronic plaque psoriasis  Trigger factors include sudden withdrawal of oral (or potent topical) corticosteroids, infections, irritating topical preparations like tar or dithranol, pregnancy and drugs

Generalised Pustular Psoriasis  Pustule swab (exclude infectious causes)  Consider skin biopsy  Admission  Fluid balance and supportive care  Bland emollients  May require systemic therapy (e.g. oral retinoid such as acitretin, Methotrexate or anti- TNF therapy)

Pemphigus Vulgaris - clinical features  Rare autoimmune blistering disorder  The blisters are intra-epidermal (therefore easily ruptured)  IgG autoantibodies against a desmosomal protein  Usually presents initially with mucosal (oral, genital, conjunctival erosions) - difficulty eating  +/- skin erosions / blisters (and positive Nikolsky sign)

Pemphigus Vulgaris - treatment  Confirm diagnosis with skin biopsy (including direct IF)  Fatal before advent of oral corticosteroids  Likely to require admission for supportive care  Non adherent dressings  High dose oral steroids initially (1 mg/kg/day)  Prevention / treatment of infection  Additional steroid sparing agent usually needed

Bullous Pemphigoid - clinical features  Autoimmune blistering disorder, commoner in the elderly  Split is at the Basement Membrane zone (deeper than in PV)  Crops of tense fluid filled blisters, often with surrounding erythema  Itchy  Can be localised or widespread  Oral mucosal involvement less frequent than PV  Usually less severe than PV

Bullous Pemphigoid -treatment  Confirm diagnosis with skin biopsy (including direct and indirect IF)  Biopsy confirmation less probable if patient already on oral corticosteroids  Admission not always necessary  Treatment – usually oral +/- topical corticosteroids (reducing course commencing around 0.5 mg/kg/day)  Attention to dressings  May need steroid sparing agent (e.g. dapsone, azathioprine)

Eczema herpeticum  Herpes simplex infections can be more severe and extensive in patients with underlying skin disease (e.g. eczema)  Systemic antivirals +/- antibiotics needed  May need admission  Ophthalmology input if eyelids involved or eyes feel gritty

Learning points  Pause before you prescribe – is this drug really necessary?  Warning signs in severe drug reaction (e.g. fever, mucosal involvement, blistering, tenderness)  Caution with oral corticosteroids in psoriasis (abrupt withdrawal can precipitate generalised pustular psoriasis)

When you need us.....  On call team (via switchboard on ) if admission needed  Call dermatology (particularly if admission avoidable but urgent treatment needed)  SPH  Ashford

…and a happy ending…… Any questions?