SYMPOSIUM: DERMATOLOGY
Management of paediatric
dermatological emergencies
Rabia Rashid
Helen Goodyear
Abstract
Most skin diseases can be safely managed in outpatients; however, in a
significant proportion of cases, immediate and aggressive intervention
is required. Recognition of these more serious and potentially life-
threatening dermatological presentations is imperative in order to pro-
vide correct emergency treatment and limit morbidity and mortality.
This article looks at conditions which may present in childhood that
might require urgent treatment. These include infection, drug reactions,
erythroderma, congenital ichthyoses (especially collodian baby and Harle-
quin ichthyosis), StevenseJohnson syndrome, toxic epidermal necrolysis,
infantile haemangiomas and epidermolysis bullosa.
Keywords adverse drug reaction; dermatology; emergency; eryth-
roderma; infection
Paediatric skin disorders account for a significant number of
cases presenting to Emergency departments or Paediatric
assessment units with around half being due to infectious causes.
Skin problems may also present in the newborn period and these
may necessitate prompt treatment. Whilst many children will
have minor conditions, or at least diseases that can be safely
treated as an outpatient a minority will present with skin mani-
festations of diseases that require urgent treatment.
Erythroderma
This is a relatively rare presentation in children, accounting for
less than 1% of paediatric dermatology clinic presentations, but
is potentially life-threatening. Erythroderma or generalised
exfoliative dermatitis is defined as an inflammatory disorder with
erythema affecting more than 90% of the body surface area. It is
often associated with generalised lymphadenopathy blistering or
scaling, Complications include hypothermia, hypernatraemic
dehydration, hypoalbuminaemia, septicaemia and high-output
cardiac failure.
In infants, hereditary ichthyoses may also present with
erythroderma. These are a large group of heterogeneous disor-
ders that are characterised by rough dry scaly skin. They include
non-bullous ichthyosiform erythroderma, bullous ichthyosiform
erythroderma, Netherton’s syndrome and ConradieHu € nermann
syndrome. Children with these conditions have a genetically
Rabia Rashid MBBS BSc MRCP is a Dermatology Specialty Registrar at
Solihull Hospital, Solihull, UK. Conflict of interest: none declared.
Helen Goodyear MB ChB FRCP FRCPCH MD MMEd MA is Head of the
Postgraduate School of Paediatrics, Health Education West Midlands,
and a Consultant Paediatrician at Birmingham Heartland Hospital,
Birmingham, UK. Conflict of interest: none declared.
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deficient skin barrier function, which predisposes them to in-
flammatory skin responses.
Immunological conditions presenting with erythroderma
include Omenn syndrome due to severe combined immunodefi-
ciency (SCID). This disorder is characterised by failure to thrive,
erythroderma, recurrent infections and lymphadenopathy.
Similar presentations occur in graft versus host disease and
hypogammaglobulinaemia.
Regardless of its cause, the treatment of erythroderma should
be prompt and vigorous. Maintaining adequate oral or parenteral
fluid intake and monitoring serum electrolytes is central to sup-
portive treatment. Attention should also be paid to wound care,
barrier nursing and thermoregulation. Topical preparations of
greasy emollients should be used liberally to hydrate the skin and
prevent fissuring. The blisters and erosions can be managed with
0.01% potassium permanganate soaks and systemic antibiotics if
necessary.
Collodian baby
This condition is slightly more common in preterm infants. A
taut, yellowish film is stretched over the skin. Eyelids and lips
may be tethered and everted (ectropion and eclabion), nasal
passages obstructed and the pinnae flattened. Constriction may
lead to digital ischaemia and fingers can appear sausage-shaped.
Typically the membrane peels away and reforms, drying out with
fissures and shedding over 1e4 weeks. Common underlying
conditions are erythrodermic or lamellar ichthyosis. Restrictive
dermopathy is an important differential diagnosis. This presents
as stiff baby syndrome with generalised taut thick tethered and
unyielding skin at birth which does not shed.
Harlequin ichthyosis
Harlequin ichthyosis is the most severe form of congenital ich-
thyosis, characterized by a thickening of the keratin layer in fetal
human skin. Thick, diamond-shaped plate-like scales are present
at birth; these restrict the movement of the infant and can lead to
contractures. In most cases mutations in the ABCA12 gene are
found. The ABCA12 gene provides instructions for making a
protein that plays a role in the transport of lipids in the
epidermis. Without sufficient quantities of this protein the skin is
thick and inflexible. Movement is restricted and splits develop in
the thick scale giving deep red fissures. Respiratory insufficiency
may be present due to restricted chest movement and also
because of prematurity.
Harlequin ichthyosis can usually be distinguished from col-
lodian baby but cases with intermediate features have been seen.
For both conditions complications include hypothermia, dehy-
dration, hypernatraemia and sepsis due to the erosions (Table 1).
Acute erythrodermic psoriasis (Figure 1)
This is rare and presents as widespread pustules on red and
tender skin. There may be a history of chronic plaque psoriasis or
triggers include infections and drugs including sudden with-
drawal of corticosteroids. Systemic symptoms may be present
including fever, headache, muscle weakness, diarrhoea and
vomiting. After a few days the pustules coalesce to form lakes of
sterile pus which dry and peel followed by successive crops.
1 Ó 2014 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: DERMATOLOGY
Treatment of collodian babies and Harlequin ichthyosis
C Humidified incubators initially transferring to a cot as soon as
possible dressing the skin with cotton tubular bandages and
greasy emollient.
C Monitor blood counts.
C Measure urea electrolytes and creatinine.
C Apply emollient to cleanse skin and ointment such as white soft
paraffin or emulsifying ointment with feeds and nappy changes.
C Ensure emollient is kept sterile.
C Beware of opportunistic infection including MRSA, Candida and
Pseudomonas and swab skin every 2e3 days.
C NG feeding may be needed for poor sucking.
C Involve an ophthalmologist in care.
C Avoid prophylactic antibiotics and placing of IV or umbilical lines
unless essential.
C Oral retinoids for Harlequin ichthyosis commenced as soon as
possible.
Table 1
Skin swabs should be taken to exclude infection and liberal
emollients applied. A skin biopsy is needed to confirm diagnosis.
Strict fluid balance and monitoring of urea, electrolytes, creati-
nine and calcium is needed. A dermatologist should be involved
in care as usually systemic immunosuppressant therapy e.g.
methotrexate, ciclosporin or acitretin is required. An important
differential diagnosis is acute generalized exanthematous pus-
tulosis (AGEP). AGEP is an acute febrile drug reaction which
Figure 1 Acute erythrodermic psoriasis.
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results in numerous small sterile pustules on erythematous skin.
Unlike StevenseJohnson syndrome there is no mucosal
involvement. Treatment of AGEP is supportive care and with-
drawal of the causative drug.
Staphylococcal scalded skin syndrome (SSSS) (Figure 2)
SSSS is an uncommon disease of early childhood. It typically
affects children less than 5 years old. It is important to differ-
entiate SSSS (synonym Ritter’s disease, pemphigus neonatorum)
from Toxic Epidermal necrolysis (TEN) and this is usually a
clinical diagnosis. The characteristic feature of skin shedding
upon light pressure (Nikolsky’s sign) is usually present but skin
biopsy can be helpful in cases where there is diagnostic doubt. In
SSSS, there is superficial separation within the epidermis, just
beneath the stratum corneum whereas in TEN there is dermo-
epidermal separation and full thickness epidermal necrosis.
The superficial nature of the skin shedding in SSSS ensures that
healing occurs without scarring. However, infants are vulnerable
to fluid loss and infection and without prompt treatment it may
be fatal.
Toxic shock syndrome (TSS)
TSS similar to staphylococcal scalded skin syndrome is caused by
exotoxins produced by Staphylococcus aureus or Streptococcus
pyogenes (Group A Streptococcus). It is an acute, multi-system
illness that leads to tissue damage, disseminated intravascular
coagulation and organ dysfunction. Even with optimal care it still
has a high mortality (5e15%) and is a diagnostic and therapeutic
challenge.
TSS can present at all ages with fever, hypotension, and
shock. Shortly after birth it is most commonly due to trans-
mission of toxigenic S. aureus from either an intrauterine infec-
tion or one acquired at the time of delivery. The extensive skin
rash initially resembles scarlet fever and can develop into
erythroderma. Rapid multi-organ failure can occur within 8e12
hours after onset of symptoms. There may be hyperaemia of the
mucous membranes and desquamation of the skin on the hands
and feet 10e21 days after disease onset.
Biochemical disturbances often include elevated creatinine,
serum transaminases and bilirubin. S. aureus is cultured in less
Figure 2 Staphylococcal scalded skin syndrome.
2 Ó 2014 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: DERMATOLOGY
than 5% of cases. Diagnosis is therefore mainly a clinical one.
Management includes prompt and aggressive repletion of intra-
vascular volume loss, oxygen and commencement of intrave-
nous antibiotics. Suitable antimicrobials for suppressing toxin
production include clindamycin and linezolid. Use of cortico-
steroids and intravenous immunoglobulins in TSS are still
controversial and a paediatric infectious disease consultant
should be consulted.
Eczema herpeticum (Figure 3a and b)
Eczema herpeticum is acute disseminated herpes simplex virus
(HSV) infection in a patient with atopic dermatitis, often asso-
ciated with systemic symptoms. Widespread cutaneous HSV
infection also occurs with other underlying dermatoses including
Figure 3 (a) Early eczema herpeticum showing vesicopustules, (b) Eczema
herpeticum showing punched-out lesions where vesicopustules have
burst.
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burns and congenital ichthyoses and in immunocompromised
children. Typically it presents as a sudden deterioration of a
child’s eczema. Lesions include vesicles, papules, crust and
punched-out lesions which tend to occur in crops and hence are
at different stages. Any cutaneous site can be involved. Associate
symptoms include fever, intense itching, malaise, vomiting,
anorexia, diarrhoea and lymphadenopathy.
Prompt recognition is essential as widespread multi-organ
involvement and failure may occur. The signs of eczema herpe-
ticum can be subtle and difficult to distinguish from bacterial
infection, especially as secondary bacterial infection usually with
S. aureus and S. pyogenes can occur in eczema herpeticum.
Treatment is supportive and with antiviral therapy. Most
children will need intravenous aciclovir in a dose of 500 mg/m2
three times daily. Children who are systemically well and have
more localised lesions as is usually the case in recurrent infection
can be treated with oral aciclovir or valaciclovir (although the
latter is unlicensed in children). Supportive treatment includes
antipyretics and analgesics, antibiotics for secondary bacterial
infection, intravenous fluids to correct dehydration and electro-
lyte imbalance and blood products if necessary. Topical steroid
ointments/creams should be discontinued as should calcineurin
inhibitors (pimecrolimus and tacrolimus) in the acute phase of
eczema herpeticum as they are thought to encourage HSV
spread.
Adverse drug reactions (ADRs)
ADRs can mimic other childhood skin diseases, especially viral
exanthems. Severe life-threatening drug reactions in children are
fortunately rare.
StevenseJohnson syndrome (SJS)
SJS and TEN are both delayed-type hypersensitivity (Type IV)
reactions and are part of the same spectrum of disease entities.
The main difference lies in the percentage of body surface area
(BSA) presenting skin detachment. In SJS, epidermal detachment
affects less than 10% of BSA, SJS/TEN overlap is when the
detachment ranges from 10% to 30%, and TEN when it affects
more than 30%.
SJS/TEN is an acute inflammatory disease, characterised by 1
e7 days of non-specific prodromal symptoms such as headache,
malaise, fever and upper respiratory tract symptoms, followed by
skin and mucosal involvement. It is usual to have at least two
mucous membrane sites involved. Common symptoms include
purulent conjunctivitis, erosions, ulcers and crusts affecting the
mouth, nose, pharynx, respiratory and digestive tract. Skin le-
sions are characteristically targetoid and progress to blisters and
epidermal detachment in sheets. As in SSSS, Nikolsky’s sign can
be positive. Visceral involvement of the kidneys, gastrointestinal
tract or liver may occur. Recovery can be slow, with the acute
episode lasting 4e6 weeks.
Causative agents for SJS primarily include drugs but also in-
fectious agents such as Mycoplasma pneumoniae. Commonly
implicated drugs are sulphonamides and anticonvulsants such as
phenobarbital, lamotrigine and carbamazepine. Supportive care
is the only universally accepted treatment for SJS. Whilst some
studies have shown that short courses of high dose corticoste-
roids have improved outcomes in SJS, others have been
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 SYMPOSIUM: DERMATOLOGY

inconclusive, shown no benefit, or even shown worse outcomes

with corticosteroids use. RegiSCAR inclusion criteria for DRESS syndrome. Three
A small number of studies and case series have explored the of the four starred criteria are required for diagnosis
use of intravenous immunoglobulin (IVIG) in SJS and TEN. Most C Hospitalisation
show favourable outcomes but others show no benefit and C Reaction suspected to be drug-related
further high quality studies are needed. Complications include C Acute rash*
loss of nails, scarring, joint contractures and a number of eye C Fever >38  C*
problems. C Lymphadenopathy in at least two sites*
SJS needs to be distinguished from erythema multiforme (EM) C Involvement of at least one internal organ*
which is a hypersensitivity reaction usually triggered by in- C Blood count abnormalities (lymphopenia or lymphocytosis*,
fections, most commonly HSV. It presents with a polymorphous eosinophilia*, thrombocytopenia*)
skin eruption characterised by macules, papules with an
abnormal centre (the typical target lesion) and plaques. Lesions Table 3
can be at different stages. In EM minor, there is very little
adults, they give an indication of severity (Table 2). One point is
mucosal involvement whereas in EM major one or more mucosal
scored for each of seven criteria present at the time of admission.
sites can be affected including the eye, mouth and genitalia.
Mortality increases with increasing score rising from more than
Involvement of an ophthalmologist is needed as eye complica-
12% for 2 points to more than 90% for 5 points.
tions can be severe. It is acute and self-limiting, usually resolving
in 2e3 weeks for EM minor and 6 weeks for EM major, without
DRESS syndrome
complications and does not progress to SJS/TEN.
DRESS (drug rash/reaction with eosinophilia and systemic
Toxic epidermal necrolysis (TEN) symptoms) is a severe drug reaction presenting with rash, fever,
lymphadenopathy, and visceral involvement, and tends to occur
TEN is almost exclusively caused by a drug eruption. Painful
2e6 weeks after initiation of an offending drug (Table 3).
tender skin with toxicity is the hallmark of the disease. The
The rash is typically a diffuse morbilliform one that may
epidermis sloughs off in large sheets that reveal a tender diffuse
progress to blisters and erythroderma. Nikolsky’s sign is absent.
erythroderma. Complications from TEN can be very severe,
Visceral involvement may include hepatitis, carditis, pneumo-
affecting the respiratory, gastrointestinal, hepatic and renal sys-
nitis, interstitial nephritis and thyroiditis. This syndrome has a
tems. Mortality is high, even with optimal care and in the liter-
10% mortality rate in the literature, most commonly from
ature ranges from 10% to 70% of cases.
fulminant hepatitis with hepatic necrosis.
Due to the potential for severe complications, patients with
Common causative drugs include anticonvulsants such as
TEN are best managed in a paediatric intensive care or burns
carbamazepine, phenobarbital, phenytoin and lamotrigine, as
unit. Withdrawal of the offending drug is essential and sup-
well as sulphonamides.
portive care includes aggressive wound care, mechanical venti-
The causative medication should be immediately withdrawn.
lation and haemodynamic support. Fluid resuscitation and
Supportive treatment includes antipyretics for fever and aggres-
nutritional care are vital due to extensive epidermal loss. Patients
sive wound care for exfoliated areas of skin. Whenever possible
should be nursed in isolation and appropriate measures taken to
admission to a burns or high dependency unit is advised. Topical
prevent infection. Ocular involvement should be anticipated and
steroids can be used for symptomatic relief but systemic corti-
an urgent ophthalmology opinion sought. Some studies have
costeroids in a tapering course over 6e8 weeks are usually
suggested that silver sulfadiazine dressings should be avoided
needed. Other immunosuppressants have been used with success
due to the association of sulphonamides with TEN. Specific
including IVIG and plasmapharesis, but there is little evidence to
treatments for TEN are controversial but have included ciclo-
justify their routine use.
sporin, plasmapheresis and TNF-a inhibitors. Patients treated
with steroids and IVIG seemed to have a better outcome. Where
possible a paediatric immunology consultant should be involved
in management. Although the SCORTEN criteria work best in

The seven SCORTEN criteria for SJS/TEN

C Age >40 years
C Presence of a malignancy (cancer)
C Heart rate >120
C Initial percentage of epidermal detachment >10%
C Serum urea level >10 mmol/litres
C Serum glucose level >14 mmol/litres
C Serum bicarbonate level <20 mmol/litres
Figure 4 Infantile haemangioma on the arm showing areas of pallor
Table 2 indicating that this is starting to resolve.

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Please cite this article in press as: Rashid R, Goodyear H, Management of paediatric dermatological emergencies, Paediatrics and Child Health
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 SYMPOSIUM: DERMATOLOGY
Purpura fulminans
Purpura fulminans is a haematological emergency that occurs in
children when there is haemorrhagic infarction of the skin and
disseminated intravascular coagulation (DIC). The most severe
form is known as acute infectious purpura fulminans which oc-
curs in severe acute sepsis. It is a cardinal feature of meningo-
coccal septicaemia. Less commonly, it is seen in Streptococcal,
Haemophilus and Staphylococcal sepsis, especially in asplenic
patients.
It can quickly progress to multi-organ failure. Cutaneous
manifestations progress rapidly from scattered petechiae to pur-
pura and ultimately necrosis and gangrene. The distal extremities
are most frequently affected, usually in a symmetrical fashion.
Meningitis, DIC and acute respiratory distress syndrome (ARDS)
are common complications.
Infantile haemangiomas (Figure 4)
The majority of infantile haemangiomas will need no treatment,
and many more do not constitute emergencies. Those which are
beginning to affect vital structures e.g. the eye or airways, napkin
area which break down easily or those which will cause a poor
cosmetic result should be urgently referred to paediatric derma-
tology. Topical timolol can be used for small flat lesions and oral
propanolol is the treatment of choice otherwise. A cardiac and
general systems examination is needed prior to commencement
of propanolol and it is helpful to consider if the child has an
associated syndrome (Table 4).
Infants with multiple small haemangiomas require a thorough
examination for cardiac or liver failure and a liver ultrasound
scan to look for haemangiomas in the liver. If present these lead
to a high mortality and are an indication for urgent treatment
with propanolol.
Epidermolysis bullosa
Epidermolysis bullosa (EB) is a group of genetic disorders that
result in skin and mucosal fragility leading to blister formation.
There are 4 main types of EB: EB simplex (EBS), junctional EB,
dystrophic EB and Kindler’s syndrome depending on the level of
Syndromes associated with haemangiomas
Phaces
C Posterior fossa brain malformations
C Haemangiomas, particularly large, segmental facial lesions
C Arterial anomalies
C Cardiac (heart) anomalies and coarctation of the aorta
C Eye abnormalities and Endocrine abnormalities
C Sternal cleft, supraumbilical raphe, or both
Pelvis
C Perineal haemangioma
C External genitalia malformations
C Lipomyelomeningocele
C Perineal haemangioma
C Vesicorenal abnormalities
C Imperforate anus
C Skin tag
Table 4
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Emergency care for a baby born with EB
C Cover all raw areas in cling film until dressings are available
C Handle carefully and avoid friction and shearing forces
C Nurse in a cot or bassinette unless premature and need an
incubator
C Do not use any adhesive dressings e soak off those which are
accidentally applied with WSP/LP 50:50
C Remove cord clamp and use a ligature instead
C Tape name band to cot or clothing
C When dressings in place dress use a soft babygro worn inside out
so that seams do not cause friction
C Use WSP/LP 50: 50 around edges of nappy
Table 5
cleavage within the skin. It is very difficult to differentiate be-
tween the types of EB and detailed information on prognosis
should not be given until biopsy results are available. Blistering
can present at birth or shortly afterwards or large raw areas may
be present especially on the lower limbs. It is important to
consider EB in the differential diagnosis of neonatal blisters
whilst excluding infectious causes (Table 5).
In England, there are two regional centres for EB based at
Birmingham Children’s hospital and Great Ormond Street. The
appropriate one should be contacted without delay for advice.
They operate an outreach service sending out dressing packs by
express courier, coming as soon as possible to the neonatal unit
to demonstrate skincare and perform the skin biopsy.
Conclusion
Paediatric dermatological emergencies can represent a diagnostic
and therapeutic conundrum for clinicians. Presentations in chil-
dren are often diverse and complex and delay in establishment of
the correct diagnosis can lead to fatal outcomes. It is important
for Paediatricians and dermatologists to be able to swiftly
recognize, diagnose and treat these conditions in children When
treating a sick child with acute skin disease, there should be a
special focus on nursing, skin and wound care, monitoring of
fluid balance and electrolytes, nutrition and early prevention and
detection of systemic complications. A
FURTHER READING
1 Aronson PL, Florin TA. Paediatric dermatologic emergencies: a case-
based approach for the paediatrician. Paediatr Ann 2009; 38:
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2 Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: recognition,
diagnosis and management. Arch Dis Child 2011; 96: 1066e71.
3 Craig T, Pu€rsu
€n EA, Bork K, et al. WAO Guideline for the management
of hereditary angioedema. WAO J 2012; 5: 182e99.
4 Ferrandiz-Pulido C, Garcia-Patos V. A review of causes of Stevens
eJohnson syndrome and toxic epidermal necrolysis in children. Arch
Dis Child 2013; 98: 998e1003.
5 Hassan I, Anwar P. Paediatric dermatological emergencies: an over-
view. J Pediatr Sci 2013; 5. e198.
6 Hoeger PH, Harper JI. Neonatal erythroderma: differential diagnosis
and management of the “red baby”. Arch Dis Child 1998; 79:
186e91.
5 Ó 2014 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: DERMATOLOGY

7 Irvine AD, Hoeger PH, Yan AC, eds. Harper’s textbook of pediatric of 64 patients managed in a specialized centre. Br J Dermatol 2013;
dermatology. 3rd edn. Oxford: Wiley-Blackwell, 2011. 169: 1304e9.
8 Lee HY, Lim YL, Thirumoorthy T, Pang SM. The role of intravenous 9 Sehgal VN, Srivastava G. Erythroderma/generalised exfoliative derma-
immunoglobulin in toxic epidermal necrolysis: a retrospective analysis titis in paediatric practice: an overview. Int J Dermatol 2006; 45: 831e9.

PAEDIATRICS AND CHILD HEALTH --:- 6 Ó 2014 Elsevier Ltd. All rights reserved.

Please cite this article in press as: Rashid R, Goodyear H, Management of paediatric dermatological emergencies, Paediatrics and Child Health
(2014), http://dx.doi.org/10.1016/j.paed.2014.10.013