Acute & Emergency Dermatology

Question 1

List several general categories of A&E Dermatology conditions

Answer 1

Erythroderma and acute skin failure
Severe drug reactions
Blistering conditions
Generalised pustular psoriasis
Eczema herpeticum
Staphylococcal scalded skin syndrome
Urticaria

Question 2

List 3 reasons for skin failure

Answer 2

Mechanical barrier to infection:
- Sepsis

Temperature regulation:
- Hypo- and Hyper- thermia

Fluid and electrolyte balance:

• Protein and fluid loss
• Renal impairment
• Peripheral vasodilation

Question 3

What is meant by Erythroderma

Answer 3

A descriptive term rather than a diagnosis

“Any inflammatory skin disease affecting >90% of total skin surface”

Question 4

List 5 conditions that cause Erythroderma

Answer 4

Causes:

1. Psoriasis
2. Eczema
3. Drugs
4. Cutaneous Lymphoma
5. Hereditary disorders

+ Unknown

Question 5

Principles of Management of A&E Dermatology conditions

Answer 5

Appropriate setting - ?ITU or burns unit
Remove any offending drugs
Careful fluid balance
Good nutrition
Temperature regulation
Emollients – 50:50 Liquid Paraffin : White Soft Paraffin
Oral and eye care
Anticipate and treat infection
Manage itch
Disease specific therapy; treat underlying cause

Question 6

Drug Reactions & A&E Dermatology conditions

Answer 6

Common
5% of inpatients
Can occur to any drug
Commonly 1-2 weeks after drug
Within 72 hours if re-challenged
Mild
- Morbilliform exanthem
Severe
- Erythroderma
- Stevens Johnson Syndrome/Toxic epidermal necrolysis
- DRESS

Question 7

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis - separate or same, which is most common?

Answer 7

2 conditions which are thought to form part of the same spectrum
Rare
1-2/million/year (SJS)
0.4-1.2/million/year (TEN)

Question 8

Casues:

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis

Answer 8

Secondary to drugs
Antibiotics
Anticonvulsants
Allopurinol
NSAIDs
Can be delayed onset

Question 9

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis

%

Answer 9

Stevens Johnson Syndrome <10%

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis 10-30%

Toxic Epidermal Necrolysis >30%

Question 10

SJS – Clinical Features

Answer 10

Fever, malaise, arthralgia
Rash 
Maculopapular, target lesions, blisters
Erosions covering <10% of skin surface
Mouth ulceration
Greyish white membrane
Haemorrhagic crusting
Ulceration of other mucous              membranes

Question 11

Toxic Epidermal Necrolysis – Clinical Features

Answer 11

Often presents with prodromal febrile illness
Ulceration of mucous membranes
Rash
May start as macular, purpuric or blistering
Rapidly becomes confluent
Sloughing off of large areas of epidermis – ‘desquamation’ > 30% BSA
Nikolsky’s sign may be positive

Question 12

Management: Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis

Answer 12

Identify and stop culprit drug as soon as possible
Supportive therapy

?High dose steroids
?IV immunoglobulins
?Anti-TNF therapy
?Ciclosporin

Question 13

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis

Prognosis - scoring system

Answer 13

Prognosis
Mortality up to 10% (SJS)/30% (TEN)
SCORTEN
Age >40
Malignancy
Heart rate >120
Initial epidermal detachment >10%
Serum urea >10
Serum glucose >14
Serum bicarbonate <20
SCORTEN
Mortality
0-1   > 3.2%
2  > 12.1%
3  > 35.3%
4   > 58.3%
5 or more  > 90%

Question 14

Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis

Long term complications

Answer 14

Pigmentary skin changes
Scarring
Eye disease and blindness
Nail and hair loss
Joint contactures

Question 15

Erythema Multiforme

Answer 15

Hypersensitivity reaction usually triggered by infection

Question 16

Erythema Multiforme causative agents

Answer 16

Most commonly HSV, then Mycoplasma pneumonia

Question 17

Erythema Multiforme - clinical features

Answer 17

Abrupt onset of up to 100s of lesions over 24 hours
Distal       proximal
Palms and soles
Mucosal surfaces (EM major)
Evolve over 72 hours
Pink macules, become elevated and may blister in centre
“Target” lesions
Self limiting and resolves over 2 weeks
Symptomatic and treat underlying cause

Question 18

(DRESS) - expand on each letter

Answer 18

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)

Question 19

DRESS
Prevalence
Clinical features

Answer 19

Incidence estimated between 1 in 1000-10,000
Mortality up to 10%
Onset 2-8 weeks after drug exposure
Fever and widespread rash
Eosinophilia and deranged liver function
Lymphadenopathy
\+/- other organ involvement

Question 20

DRESS

Treatment

Answer 20

Stop causative drug
Symptomatic and supportive
Systemic steroids
+/- Immunosuppression or immunoglobulins

Question 21

Pemphigus – Clinical features

Answer 21

Antibodies targeted at desmosomes
Skin – flaccid blisters, rupture very easily
Intact blisters may not be seen
Common sites – face, axillae, groins
Nikolsky’s sign may be +ve

Question 22

Pemphigus – Clinical Features

Answer 22

Commonly affects mucous membranes
Ill defined erosions in mouth
Can also affect eyes, nose and genital areas

Question 23

Pemphigus - Pathology/investigation

Answer 23

Histopathology
epidermal-dermal tears
Immunofluorescence - more widespread around cells

Question 24

Pemphigoid - Pathology/investigation

Answer 24

Antibodies directed at dermo-epidermal junction
Intact epidermis forms roof of blister
Blisters are usually tense and intact

Question 25

Pemphigoid

Answer 25

Pemphigoid
Common
Elderly patients
Blisters often intact and tense
Even if extensive, patients are fairly well systemically
Topical steroids may be sufficient if localised; systemic usually required if diffuse

Question 26

Pemphigus

Answer 26

Pemphigus
Uncommon
Middle aged patients
Blisters very fragile – may not be seen intact
Mucous membranes usually affected
Patients may be very unwell if extensive
Treat with systemic steroids. Dress erosions. Supportive therapies

Question 27

Erythrodermic psoriasis and Pustular Psoriasis

Answer 27

Can occur without previous history of psoriasis
Common causes:
Infection
Sudden withdrawal of oral steroids or potent topical steroid
Rapid development of generalised erythema, +/- clusters of pustules
Fever, elevated WCC
Exclude underlying infection, bland emollient, avoid steroids
Often require initiation of systemic therapy

Question 28

Eczema Herpeticum

Answer 28

Disseminated herpes virus infection on a background of poorly controlled eczema
Monomorphic blisters and “punched out” erosions
Generally painful, not itchy
Fever and lethargy
Treatment dose Aciclovir
Mild topical steroid if required to treat eczema
Treat secondary infection
Ophthalmology input if peri-ocular disease
In adults consider underlying immunocompromise

Question 29

Staphylococcal Scalded Skin Syndrome

Answer 29

Common in children, can occur in immunocommpromised adults
Initial Staph. infection
May be subclinical
Diffuse erythematous rash with skin tenderness
More prominent in flexures
Blistering and desquamation follows
Staphylococcus produces toxin which targets Desmoglein 1
Fever and irritability
Require admission for IV antibiotics initially and supportive care
Generally resolves over 5-7 days with treatment

Question 30

Urticaria

Answer 30

Weal, wheal or Hive:
Central swelling of variable size, surrounded by erythema. Dermal oedema
itching, sometimes burning
Histamine release into dermis
fleeting nature, duration: 1- 24 hours
Angioedema
Deeper swelling of the skin or mucous membranes

Question 31

Acute Urticaria (< 6 week History)
Causes

Answer 31

Idiopathic
50%
Infection, usually viral
40%
Drugs, IgE mediated
9%
Food, IgE mediated 
1%

Question 32

Acute Urticaria (< 6 week History)
Treatment

Answer 32

Oral antihistamine
Taken continuously
Up to 4 x dose
Short course of oral steroid may be of benefit if clear cause and this is removed
Avoid opiates and NSAIDs if possible (exacerbate urticaria)

Question 33

Chronic Urticaria (> 6 week history)

Answer 33

Autoimmune/Idiopathic
60%
Physical
35%
Vasculitic
5%

Rarely a Type 1 hypersensitivity reaction

Question 34

Management of Chronic Urticaria

Answer 34

1. Standard dose non-sedating H1 antihistamine
2. Higher dose H1 Antihistamine 4x recommended dose
   or + second antihistamine
3. Consider 2nd line agent, anti-leukotriene or, if angioedema present use tranexamic acid.
   Limited role for oral steroids. No good evidence base.
4. Consider an immunomodulant
   (e.g. omalizumab, cylosporine)

Omalizumab
Monoclonal antibody to IgE, mechanism of action unknown
Recently licensed for use in chronic spontaneous urticaria
300mg S/C ever 4 weeks
£6000 per year per patient

Question 35

A&E Dermatology key points to remember

Answer 35

Skin is an organ - It can fail just like any other
Take a good drug history!
Principles of management of skin failure are the same regardless of cause
Good fluid and electrolyte balance
Temperature regulation
Emollients
Anticipate and treat infection