Dermatological Emergencies Flashcards

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1
Q

What is erythroderma?

A

Intense and widespread reddening of the skin due to inflammation - can also be known as exfoliative dermatitis. Idiopathic erythroderma is sometimes called ‘red man syndrome’.

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2
Q

What are common causes of erythroderma?

A
  • Drug eruption: allopurinol, abx, antiepileptics, Ca channel blockers, opiates, isoniazid, thiazides
  • Dermatitis, especially atopic dermatitis
  • Psoriasis, especially after withdrawal of systemic steroids
  • Pityriasis tubra pilaris (group of rare skin disorder that present with reddish orange coloured scaling patches with well-defined border)
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3
Q

What other skin diseases less frequently cause erythroderma?

A
  • Contact dermatitis, stasis dermatitis (venous eczema) and in babies, seborrhoeic dermatitis or staphylococcal scalded skin
  • Pemphigus or bullous pemphigoid
  • Sezary syndrome (erythrodermic form of cutaneous T cell lymphoma)
  • Several rare congenital ichthyotic conditions
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4
Q

What are signs and symptoms of erythroderma?

A
  • Warm skin
  • Itchy with lichenification
  • Eyelid swelling can cause ectropion
  • Scaling starts 2-6 days after onset of erythema as fine flakes or large sheets
  • Thick scaling on scalp with hair loss or even complete baldness
  • Swollen lymph nodes
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5
Q

What clues can be present in erythroderma for identifying the underlying cause?

A
  • Serous ooze - clothes and dressings stick to skin and an unpleasant smell - atopic erythroderma
  • Persistence of circumscribed scaly plaques in certain sites like elbows and knees - psoriasis
  • Islands of sparing, follicular prominence, orange-hue to keratoderma - pityriasis rubra pilaris
  • Subungual hyperkeratosis, crusting on palms and soles and burrows - crusted scabies
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6
Q

What systemic symptoms can be present in erythroderma?

A
  • Lymphadenopathy, hepatosplenomegaly, abnormal liver dysfunction and fever - may suggest drug hypersensitivity syndrome or malignancy
  • Leg oedema may be due to inflamed skin, high output cardiac failure (heart is pumping normal amount of blood, but it is not enough to help the body work as it should) +/or hypoalbuminaemia
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7
Q

What are the complications of erythroderma?

A
  • Heat loss - hypothermia
  • Fluid loss - electrolyte imbalance and dehydration
  • Red skin - high output cardiac failure
  • Secondary skin infection - impetigo, cellulitis
  • General unwellness - lead to pneumonia
  • Hypoalbuminaemia - due to protein loss and increased metabolic rate causes oedema
  • Longstanding erythroderma may result in pigmentary changes (brown +/or white skin patches)
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8
Q

What is impetigo?

A

Bacterial infection of superficial skin, presents as yellow crusts on face, arms or legs

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9
Q

How is erythroderma diagnosed?

A
  • > 20% of circulating sezary cells suggest sezary syndrome (aggressive form of cutaneous T cell lymphoma - group of disorders that occur when T cells become cancerous and affect the skin)
  • Proteins may show hypoalbuminaemia and abnormal LFTs
  • Polyclonal gamma globulins are common and raised IgE is typical of idiopathic erythroderma
  • Skin biopsies may be taken if cause is unknown, tends to show non-specific inflammation on histopathology
  • Direct immunofluorescence is of benefit if an autoimmune blistering disease or CTD is considered
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10
Q

What is the treatment for erythroderma?

A
  • Discontinue all unnecessary medications
  • Monitor fluid balance and body temperature
  • Maintain skin moisture with wet wraps, other types of wet dressings, emollients and mild topical steroids
  • Abx for bacterial infection
  • Antihistamines for itch
  • If cause can be identified then specific treatment should be started, such as topical and systemic steroids for atopic dermatitis, acitretin or methotrexate for psoriasis
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11
Q

What is SJS/TENs?

A

Severe skin reaction typically triggered by specific medicines. SJS is the less severe form of the disease (10% of skin affected), TENs is the most severe form (>30% of skin affected). It is a type 4 hypersensitivity reaction. Both the mucosal lining and skin affected.

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12
Q

What are the signs and symptoms of SJS/TENs?

A
  • Fever
  • Early: flu-like symptoms - sore throat, cough, red eyes, tender pink skin
  • Macule, dark red centre, shaped lesions, also fluid filled vesicles, may get flaccid bullae
  • Skin starts peeling off, leaving painful raw areas called erosions (resembles hot water burns)
  • Rubbing skin can cause peeling - Nikolysky’s sign
  • Leads to - fluid loss, dehydration, pneumonia, sepsis, shock, multiple organ failure
  • Diagnosis is made on symptoms, but biopsy can give definitive diagnosis
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13
Q

What are the long term effects of SJS/TENs?

A
  • Changes in skin colouring (pigmentation), dryness of skin and mucous membranes (xerosis), excess sweating (hyperhidrosis), hair loss (alopecia) and abnormal growth/loss of finger nails and toenails
  • Other chronic problems - impaired taste, difficulty urinating and genital problems
  • A minority develop throat dryness/inflammation of eyes which can lead to increased sensitivity to light (photophobia) and vision impairment
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14
Q

What are causes of SJS/TENs?

A
  • HLA-B gene most commonly associated with SJS/TENs
  • In a process not well understood, the medication causes cytotoxic T cells and NK cells to release a substance called granulysin that destroys cells in the skin and mucous membranes
  • Certain medications
  • Pneumonia due to mycoplasma pneumonia and viral infections (including CMV) has caused SJS/TENs
  • Cause not fully understood
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15
Q

What medications can cause SJS/TENs?

A
  • Seizure medications (carbamazepine, lamotrigine, phenytoin etc)
  • Allopurinol
  • Antibiotic class - penicillin, sulphonamides
  • Nevirapine (treats HIV)
  • Sulfasalazine (treats IBD)
  • Oxicams (type of NSAID -cam)
  • OCP
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16
Q

What are differentials for SJS/TENS?

A
  • Morbilliform drug rash
  • Erythema multiforme
  • Drug induced linear IgA disease
  • Acute graft vs host disease
  • Drug reaction with eosinophilia and systemic symptoms syndrome
  • Staphylococcal scalded skin syndrome
17
Q

What is the general management for SJS/TENS?

A
  • Treated in burns unit or ICU
  • Stop all suspected drugs
  • Give antihistamines: IVIG, steroids - suppress immune system
  • Monitor fluid losses + temp
  • Monitor electrolytes
  • Sterile wound care
  • Skin biopsy: a drug reaction causes a series of immune responses leading to attack on the keratinocytes - full thickness necrosis of the epidermis, there is minimal inflammation
18
Q

What are the management steps in relation to the function of the skin?

A
  • Regulation (barrier to prevent loss of fluid): monitor pulse and BP; fluid replacement, catheterise, emollients/non-occlusive dressings
  • Regulation (temperature homesostasis): warm environment
  • Protection (infection): monitor temperature, sterile environment and antiseptic
  • Sensation: analgesics; gentle handling
19
Q

What other aspects of the body need care in SJS/TENS?

A
  • Nutritional care: consider NG feed
  • Eye care: frequent eye drops/ointments (antiseptic, antibiotic, cortisone)
  • Mouth care: mouthwashes, topical oral anaesthetic
  • Genital care: emollients, gynae review
  • Lung care: humidify air, physiotherapy, ?intubation and mechanical ventilation if trachea and bronchi are involved
20
Q

What is the definition of Steven-Johnson Syndrome (SJS)?

A

Skin detachment <10% of body surface area (BSA), widespread erythematous or purpuric macules or flat atypical target.

21
Q

What is the definition of Steven-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TENS) overlap?

A

Detachement between 10-30% of BSA, widespread purpuric macules or flat atypical targets

22
Q

What is the definition of TENS with spots?

A

Detachment >30% of BSA, widespread purpuric macules or flat atypical targets

23
Q

What is the definition of TENS without spots?

A

Detachment >10% of BSA, large epidermal sheets and no purpuric macules

24
Q

What are causative medications for TENS?

A
  • Anticonvulsants e.g. carbamazepine, phenytoin, valproate, lamotrigine
  • Antibiotics e.g. sulphonamides, also aminopenicillins, quinolones, cephalosporins
  • Antivirals (HAART)
  • Antifungals (imidazoles)
  • Antimalarials
  • Antiretrovirals e.g. nevirapine, abacavir
  • Anti-inflammatories - NSAIDs (oxicams in particular)
  • Analgesics e.g. paracetamol
  • Allopurinol
  • Anti-cancer drugs e.g. bendamustine, chlorambucil
  • Infectious agenets (i.e. mycoplasma pneumonial, herpes virus, Hep A) and immunisations
25
Q

What are the causes of deaths due to TENS?

A
  • Septic shock
  • Multisystem organ failure
  • Epithelial loss: vulnerability to bacterial and fungal infections
  • Sloughing of stratified epithelium of MMS: GI haemorrhage, respiratory failure, eye and GU abnormalities
  • Fluid loss from extensive skin exfoliation and an inability to eat/drink: hypovolaemia, acute tubular necrosis + shock
26
Q

What are the criteria for SCORTEN?

A

1 point for each of the 7 criteria present:

  • Age <40yrs
  • Presence of malignancy
  • HR >120
  • Initial % of epidermal detachment >10%
  • Serum urea level >10mmol/l
  • Serum glucose level >14mmol/l
  • Serum bicarbonate level <20mmol/l
27
Q

What do scores from SCORTEN mean?

A

Mortality rate:

  • SCORTEN 0-1: >3.2%
  • SCORTEN 2: >12.1%
  • SCORTEN 3: >35.3%
  • SCORTEN 4: >58.3%
  • SCORTEN 5 or more: >90%
28
Q

What are the complications for TENS?

A
  • Eye: dry eye (corneal abrasions/scarring), palpebral synechiae, entropin or symblepharon (adhesion of the eyelids). 5-9% of patients can become blind.
  • Other MM strictures due to scarring: chronic xerostomia, oesophageal strictures, vulvovaginal synechiae, phimosis. Also chronic erosion of mouth and genitalia
  • Lung disease e.g. bronchiolitis, bronchiectasis
  • Nails: loss and dystrophy
  • Skin: dermal desiccation, causing deep dermal wounds, inability to sweat, scarring, underlying joint contractions, dyspigmentation
29
Q

What is the pathophysiology of Staphylococcal Scalded Skin Syndrome?

A

Staph A releases 2 toxins:
- Exfoliative A: causes localised infection, bullous impetigo
- Exfoliative B: causes SSSS
The toxin attacks the epidermal layer of skin (specifically desmoglein 1) - affects stratum spinosum and granulosum

30
Q

What are the signs and symptoms of bullous impetigo?

A

Blisters (look like honey coloured vesicles) on top of erythematous base

  • Honey coloured blisters can also be caused by streptococcus pyogenes
  • Primarily found in exposed areas and orificies e.g. mouth
  • Can rupture (usually become cloudy before rupturing)
  • Diagnosis: can aspirate fluid then gram stain
31
Q

What are the signs and symptoms of Staphylococcal Scalded Skin Syndrome (SSSS)?

A
  • Mainly perioral area
  • Flexures - much darker in these areas
  • Very tender
  • Nikolysky sign positive
  • Fever
  • Generally heals within 5-7 days (faster with abx)
  • Generally there’s a specific area where the bacteria is residing e.g. may have purulent conjunctivitis, naspharyngeal infection (so they’ll probably have a localised infection before developing SSSS)
32
Q

What are differentials for SSSS?

A
  • SJS/TENS: usually in younger people, history of drug use 8 weeks ago
  • Tzanck smear: acantholytic lesions in SSSS but not SJS/TENS
33
Q

What are the treatments for SSSS?

A
  • Emollients
  • Fluids and electrolytes (IV)
  • Antibiotics
  • Dressings