Skin conditions are common in children and can vary widely in severity. Numerous dermatoses can present to the paediatric emergency department; few have a high mortality and many may occur at any age.
There are major global differences in the epidemiology of paediatric skin disorders, but at least 10% of children will experience angiomas, warts or atopic dermatitis.1
The skin can also manifest features of other serious conditions, such as ecthyma gangrenosum in pseudomonas sepsis or Janeway lesions and Osler's nodes in endocarditis, conditions that should be recognised and acted on promptly.
It is important to note that skin involvement is not always proportional to the severity of the underlying pathology. For example, viral exanthems in children usually represent a minimal health risk, whereas minimal skin exfoliation in staphylococcal scalded skin syndrome (SSSS) can be associated with severe underlying inflammation.
Infections are the cause of most acute paediatric emergency presentations, some of which are discussed in this article. Box 1 summarises some potentially threatening conditions that should also be recognised and acted on promptly.
|BOX 1: DANGEROUS SKIN CONDITIONS|
|Purpura fulminans||Meningococcus, pneumococcus, varicella||Extensive necrotic purpura, hypotension, intravascular disseminated coagulopathy|
|Multiple abscesses||Staph aureus (?MRSA)||Multiple necrotic abscesses, necrotising pneumopathy|
Staph aureus, Streptococcus pyogenes
|Scarlet fever-like skin rash, fever, hypotension, vomiting, diarrhoea, multi-organ failure|
|SSSS||Staph aureus||Skin exfoliation, leaving raw-looking, tender erosions, systemic symptoms|
|Cellulitis||Staph aureus, Strep pyogenes||Warm, tender erythema with oedema, fever|
|Kaposi varicelliform eruption (eczema herpeticum)||Human herpes virus type 1 (?HHV-2)||Widespread vesicular herpetic eruption in a patient with pre-existing eczema|
|Sulfonamides, anticonvulsants, allopurinol etc||Purpuric macules progressing to blisters and areas of epidermal necrosis (targetoid appearance) with
mucosal involvement, fever
and malaise. Extensive skin
|Drug reaction with eosinophilia and
|Carbamazepine, allopurinol, etc||Severe eruption, fever, hypereosinophilia, internal organ dysfunction|
|Fever, desquamation at extremities, polymorphous exanthema, bilateral bulbar conjunctivitis, strawberry tongue, cervical
|Periungual telangiectasia, photosensitive rash, internal organ involvement (lupus), proximal muscle weakness (dermatomyositis)|
|Haematological disorders||Purpura, lymphadenopathy|
Children presenting with alarming signs, such as purpura, skin pain, mucosal involvement or internal organ dysfunction, should be carefully assessed. Detailed history-taking about the chronology of symptoms and having a high index of suspicion are crucial to the diagnosis.
Purpura fulminans (PF) is diagnosed when there is sudden cutaneous haemorrhage and necrosis.2 It is classified as neonatal, idiopathic or acute infectious PF, depending on the trigger mechanism.
Neonatal PF usually presents within the first few days of life. Purpuric lesions can affect the torso, perineal area or thigh flexor regions. Several causes have been reported, including protein C mutations and inherited deficiency of protein S or antithrombin III.3
Bacterial or viral illnesses can lead to the development of idiopathic PF within seven to 10 days of the start of the infection, of which streptococcus and varicella are the most common.4
Acute infectious PF, the most common variant, is usually a result of an underlying bacterial infection. Meningococcus and varicella are the most common causative organisms, but staphylococci and Gram-negative bacilli have also been reported.3 Bacterial endotoxins can lead to the imbalance of proand anticoagulant endothelial activity during illness, affecting proteins C and S and antithrombin III levels.5
In all three categories of PF, there may be extensive ecchymosis of the skin, alongside gangrenous lesions that are non-blanching, with features suggestive of disseminated intravascular coagulation.6 In later stages, bullae may herald the onset of haemorrhagic necrosis. The extremities are often most severely affected, with formation of firm eschar and slough.
Haematology laboratory results will commonly show reduced levels of clotting factors, fibrinogen and platelets, with prolonged bleeding times.7 As expected, levels of antithrombin III and proteins C and S would also be reduced.
Supportive measures, fluid resuscitation, antibiotics and volume expansion remain key to managing this condition. Blood products and clotting factors may also be replaced.
Skin necrosis development may be reversed with the use of heparin, because this bonds with antithrombin III, preventing thrombus formation.7
Fresh frozen plasma and cryoprecipitate can be helpful in patients with homozygous protein C deficiency, as this has been shown to improve survival.8 Disseminated intravascular coagulation may also be reversed with the replacement of antithrombin III.8
Surgical excision of necrotic tissue has been recommended and escharotomies may be prudent.9 Peripheral perfusion may be improved with the use of recombinant tissue plasminogen activator, which also enhances fibrinolysis.10
Staphylococcal scalded skin syndrome
SSSS is a rare syndrome, typically seen in infants and young children. It occurs in individuals infected or colonised with strains of Staphylococcus aureus that secrete exfoliative exotoxins.11 Phage group II Staph aureus is the most commonly isolated strain. Overall, only about 5% of all Staph aureus strains produce these toxins.11
SSSS usually affects children under the age of five years, probably because they lack antibodies against exfoliative toxins. It has been shown that antibodies to these toxins are found in only 30% of children aged three to 24 months, compared with 91% of adults aged over 40 years.12
After a short prodromal period, tender and reddened areas appear on the skin. Circulating exotoxins precipitate a severe inflammatory reaction and lead to superficial skin separation underneath the corneal layer, resulting in flaccid blisters that rupture easily, leaving tender, raw-looking erosions, which resemble scalded skin (see box 2).
|BOX 2: CLINICAL PRESENTATION OF SSSS13|
Within 48 hours
Tender reddened patches on face, neck, axillae, perineum
|Flaccid bullae develop within reddened areas|
|Bullae rupture easily and reveal moist, reddened base (scalded appearance)|
|Skin lesions often heal over seven to 10 days without scarring|
Patients are unlikely to have SSSS more than once, although recurrent episodes have been reported.13,14 Hypothermia and sepsis are two recognised complications.
Diagnosis is usually clinical. Tender erosion in children is a useful diagnostic feature. In cases where it is difficult to make a diagnosis, a biopsy for histology may show intra-epidermal cleavage through the granular layer. Immunofluorescence studies can rule out other blistering skin conditions.
It is also useful to isolate the bacteria producing the exotoxin by microbiological studies. Swabs should be taken from the probable primary infection site and common colonisation areas, usually the nares of the nose, the throat, axillae and umbilicus. PCR on serum samples can show the presence of the toxin.15
It is crucial to recognise this condition early and treat it with parenteral antistaphylococcal antibiotics, such as flucloxacillin. If treated promptly, SSSS resolves in days and the lesions heal without scarring. In cases where patients have MRSA, there is a higher risk of mortality. Glycopeptides may be considered as alternatives in this case.16
As the disease process only affects the epidermis, the haemodynamic, fluid and electrolyte imbalance are not as severe as in Stevens-Johnson syndrome or toxic epidermal necrolysis (TEN). However, when the skin involvement in SSSS covers several body sites and the patient is persistently unwell, more intensive care should be arranged.
This should include regular monitoring of core body temperature and replacement of fluid and electrolytes, as in burns patients.13,15 Aseptic wound care and pain management should be initiated.
Kawasaki disease is an acute multisystem vasculitic disease of unknown aetiology. It is the most common cause of acquired cardiovascular disease among children.17
It is hypothesised that Kawasaki disease develops as a result of spontaneous triggering of the immune system by an environmental agent.18
Various causative agents have been linked, including Rickettsia, humoral factors, spirochetes, retroviruses and bacterial antigens. IgA plasma cells have also been isolated at the mucosal surfaces of those with the disease – a finding in most children with infectious disease.19
Up to 75% of reported cases occur in children under the age of five years, with a steadily rising incidence worldwide.20 There is evidence of a genetic predisposition, where siblings affected by Kawasaki disease are 10 times more likely to develop the condition than the normal population. Studies have shown that up to a third of patients may have a simultaneous infection at the time of diagnosis, although no link has been established between severity and specific agents.21
There is a similarity, clinically and biochemically, between Kawasaki disease and toxic shock syndrome and it is therefore important to differentiate between the two. There are four phases in the development of Kawasaki disease (see box 3).
|BOX 3: DEVELOPMENT OF KAWASAKI DISEASE|
Four developmental phases
|Acute Recurrent spiking of temperature up to 40°C and cardiac symptoms such as pericarditis – this is the ideal time for diagnosis to prevent long-term complications|
|Subacute Two weeks after fever abates, when there is the greatest risk of sudden cardiac death|
|Convalescent Symptom-free period while biochemical results return to normal level|
|Chronic The follow-up period, where patients with cardiac involvement are monitored long-term|
The exanthem seen in Kawasaki disease presents as a truncal eruption and may be described as morbilliform, similar to the eruption in measles. Characteristically, it starts at the perineum and progresses to desquamation. This process may be more rapid than the finger peeling, which may occur two weeks after onset. There is no validated diagnostic laboratory test in Kawasaki disease22 and diagnosis is based on clinical criteria adapted by the American Heart Association.17 Before the optimisation of medication, mortality rates of up to 2% had been reported.23
The acute management of Kawasaki disease focuses on reducing inflammation, as in myocarditis and coronary arteritis.24 IV immunuglobulin remains the first-line treatment, despite the aetiology of the disease not being fully understood.
Several multicentre prospective trials have shown the efficacy of IV immunoglobulin.25 The American Heart Association recommends a 12-hour infusion at a dose of 2g per kg, administered within 10 days of the onset of fever, as well as aspirin as an antiplatelet until the fever abates.17 Recent studies have shown that the addition of IV steroid may prevent the development of severe coronary disease.26
It is recommended that patients have regular follow-ups with paediatric cardiologists, who will be able to monitor aneurysm and cardiac disease progression by echocardiogram, as well as stress and perfusion heart scans.27
- Dr Faraz Ali is a clinical research fellow and Dr Ausama Atwan is a clinical lecturer at Cardiff University. Dr Ru Katugampola and Dr Manju Kalavala are both consultants in paediatric dermatology at the University Hospital of Wales
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