Dermatological Emergencies Flashcards

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1
Q

What is erythroderma?

A

widespread inflammation affecting 90% or more of the body surface

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2
Q

What are the symptoms of erythroderma?

A
Thirst
Fever
Chills
Malaise
Diziness
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3
Q

Generalised lymphadenopathy can occur in erythroderma?

A

Yes - especially in erythroderma caused by underlying malignancy

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4
Q

List some common causes of erythroderma?

A
Atopic eczema
Psoriasis
Drugs - sulphonamides, gold, sulphonylureas, penicillins
Seborrhoeic eczema
Idiopathic
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5
Q

List some rare causes of erythroderma?

A
Chronic actinic dermatitis
Cutaneous T cell lymphoma
Malignancy - especially leukaemia
Pemphigus foliceus 
Pityriasis rubra pilaris
HIV infection
Toxic shock syndrome
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6
Q

What are the complications of erythroderma?

A

High output cardiac due to increased blood flow
Hypothermia from heat loss
Pre-renal acute kidney injury due to fluid depletion
Hypoalbuminaemia
Catabolism
Secondary bacterial infection
Capillary leak syndrome

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7
Q

Describe capillary leak syndrome

A

Here the release of inflammatory mediators from the skin causes extensive vascular leakage which can lead to oedema, hypovolaemic shock and acute respiratory distress syndrome

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8
Q

Describe the management of erythroderma?

A

Supportive treatment - maintain body temperature (space blankets, heaters) and close monitoring
Skin swabs if secondary infection suspected
Stop non-essential medication
Topical therapy with bland emollient or mild topical steroids while underlying cause is identified
Treat underlying cause

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9
Q

What are the different types of skin drug reactions?

A
Maculopapular rash
Urticaria
Papulosquamous rash
Phototoxic rash 
Pustular rash
Lichenoid rash
Fixed drug rash
Bullous rash
Itch
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10
Q

List some common drugs which cause acute rashes

A
Antibiotics - penicillins, trimethoprim
NSAIDs
Chemotherapeutic agents
Psychotropic agents
Anti-epletic agents 
Cardiac drugs - beta blockers, ACE inhibitors, anticoagulants
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11
Q

Where do maculopapular drug rashes usually occur?

A

On the torso and spread to the face and limbs but spare the mucosae

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12
Q

Maculopapular drugs rashes are self limiting and usually clear within how many weeks?

A

1-2 weeks

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13
Q

What conditions are classed as severe drug rashes?

A

Stevens Johnson syndrome and toxic epidermal necrolysis
Drug reaction with eosinophilia and systemic symptoms
Acute generalised exanthematous pustulosis
Serum sickness like reactions

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14
Q

What drugs are common causes of severe cutaneous drug reactions?

A
Antibiotics
NSAIDs
Anticonvulsants
Allopurinol
Dapsone
Nevirapine
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15
Q

Define Steven johnson syndrome

A

A severe mucutaneous disorder characterised by blistering, epidermal detachment and mucosal ulceration
SJS occurs when there is <10% skin detachment and one/two mucosal sites involved

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16
Q

What is Steven Johnson syndrome and toxic epidermal necrolysis overlap syndrome?

A

When there is 10-30% skin detachment

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17
Q

At what point does disease progress to toxic epidermal necrolysis?

A

when there is >30% skin detachment and all mucosal sites are involved

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18
Q

Describe the pathophysiology of Steven Johnson syndrome and toxic epidermal necrolysis?

A

Cytotoxic T cells attack epithelial cells in the mucosa and epidermis in response to a drug antigen and release perforin (which lysis target epithelial cells) and granzymes (which induces necrosis of epithelial cells). T cells are release pro-inflammatory cytokines which attract other immune cells
This leads to a loss of skin barrier protection, erosion of the affected skin and mucosa and detachment at the basement membrane

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19
Q

Which medications most commonly cause Steven Johnson syndrome and toxic epidermal necrolysis?

A

Antoconvulsants
Antibiotics
NSAIDs

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20
Q

Other than drugs, what. can cause Steven Johnson syndrome and toxic epidermal necrolysis?

A

Mycoplasma pneumonias infection

Cytomegalovirus infection

21
Q

How long after drug exposure does Stevens Johnson syndrome / toxic epidermal necrolysis usually occur?

A

1-2 weeks

22
Q

What are the symptoms fo Steven Johnson syndrome / toxic epidermal necrolysis?

A

Initial non-specific symptoms of malaise, myalgia, fever and cough
Formation of tender maculopapular lesions on the torso and mucosal surfaces

23
Q

What scoring system can be used to estimate prognosis in Steven Johnson syndrome / toxic epidermal necrolysis?

A

SCORTEN

24
Q

How is Steven Johnson syndrome / toxic epidermal necrolysis managed?

A

High dose steroids
Ciclosporin
IV immunoglobulin therapy
Stop possible causative. drugs

25
Q

How many weeks after drug exposure does drug reaction with eosinophilia and systemic symptoms usually occur?

A

2-6 weeks

26
Q

Describe the presentation of drug reaction with eosinophilia and systemic symptoms?

A

Widespread erythema, facial oedema, fever, lymphadenopathy and hepatosplenomegaly

27
Q

What drugs are a common cause of drug reaction with eosinophilia and systemic symptoms?

A

Anticonvulsants

28
Q

How is drug reaction with eosinophilia and systemic symptoms managed?

A

Systemic steroids

29
Q

Where does the small, non-follicular sterile pustules usually form in acute generalised exanthematous pustulosis?

A

Neck
Axillae
Groin

30
Q

How is acute generalised exanthematous pustulosis treated?

A

Topical steroids and emollients

31
Q

What is erythema multiforme?

A

A self-limiting, symmetrical rash characterised by target lesions on the distal limbs, palms and soles

32
Q

Describe the characteristic appearance of the rash in erythema multiforme?

A

Concentric rings of erythema with a dusky centre, which may occasional blister
Vesicles and papule may be present

33
Q

Erythema multiforme can affect mucous membranes. T/F?

A

True - but only in erythema multiforme major

34
Q

What type of hypersensitivity reaction is erythema multiforme?

A

Type IV hypersensitivity reaction

35
Q

What is the most common trigger for erythema multiforme?

A

Herpes simplex virus

36
Q

Describe the management of erythema multiforme?

A

Supportive with analgesia and fluids (possibly IV)

37
Q

What is immunobullous disease?

A

An autoimmune disease which targets antigens in keratinocytes and the basement membrane to cause skin blistering

38
Q

What investigations can be used for immunobullous disease?

A

Light and electron microscopy

Immunofluorescence

39
Q

Describe the difference between direct and indirect immunofluorescence testing for immunobullous disease?

A

Direct - investigates the presence of antibody deposition in affected skin
Indirect - investigates the presence of circulating skin autoantibodies

40
Q

In which ethnic groups is pemphigus vulgaris most common?

A

Ashkenazi Jews

People from the Indian subcontinent

41
Q

Describe the pathophysiology of pemphigus vulgaris?

A

IgG4 antibodies attack desmosomal proteins causes a loss of keratinocyte adhesion in the skin and mucous membranes

42
Q

Where does the rash commonly occur in the pemphigus vulgaris?

A

Torso

43
Q

Where does the rash commonly occur in the pemphigus foliaceus?

A

Seborrhoeic areas e.g. scalp, face and upper chest

44
Q

How is pemphigus vulgaris and pemphigus foliaceus treated?

A

High dose systemic corticosteroids

Immunosuppressants e.g. azathioprine, cyclophosphamide, methotrexate, ciclosporin

45
Q

What age group is most commonly affected by bullous pemphigoid?

A

Patients >60 years

46
Q

Describe the pathophsyiology of bullous pemphigoid?

A

There are large, tense, serous or haemorrhage blisters (bullae) which can appear anywhere on the body but especially on the limbs and torso

47
Q

Pemphigoid can be very itchy. T/F?

A

True

48
Q

How is bullous pemphigoid treated?

A

Oral corticosteroids
Long term steroid sparing agents e.g. azathioprine, mycophenolate mofetil
Topical steroid