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Education & Practice Online First, published on March 7, 2018 as 10.1136/archdischild-2017-313998
Best practice
1
Emergency Department, Case scenario Of these infections, MD is arguably the
Royal Belfast Hospital for Sick
It's 2am and you are called to review a ‘well- one we worry about most in the UK but
Children, Belfast, UK
2
Paediatric Department, Royal looking child’ in the emergency department how commonly is MD responsible for a
Free London NHS Foundation who has presented with a new non-blanching NBR in a child?
Trust, London, UK
3
rash. He has been hot at home with some This is surprisingly difficult to answer
Emergency Department, Bristol
Royal Hospital for Children,
coryzal symptoms. Mum is worried, she because studies are difficult to compare
Bristol, UK thinks the rash has spread in the last hour! due to their heterogeneity. For example,
4
Faculty of Health and Applied one study looking at all presentations of
Sciences, University of the West What are you going to do? fever (>38°C) and NBR presenting to the
of England, Bristol, UK
In this article, we discuss the aetiology and ED found that only 1% of children had
Correspondence to
initial assessment of non-blanching rashes in MD as the cause.2 In contrast, studies of
Dr Thomas Waterfield; thomas. children. hospitalised children with fever and NBR
waterfield@googlemail.com have reported rates as high as 23%.3
Introduction
Received 7 November 2017 Non-blanching rash (NBR) is a term for any
What about other serious bacterial infections?
Revised 23 January 2018 rash in which the colour is unchanged with
Accepted 4 February 2018 Other invasive bacterial infections may
direct pressure. The presence of a NBR is of
also present with a NBR—especially
concern to both parents and clinicians as it is
streptococcus infections.1 4 6 Other infec-
associated with a wide range of underlying
tious causes are rare (<1% of cases).1–3
diagnoses, some of which are life threat-
ening. The term is usually used to refer to Mechanical causes
the presence of petechiae/purpura (figure 1), A mechanical cause is identified in almost
and in this form it is a relatively common a quarter of NBR in children,3 the most
presentation to the emergency department common being straining, coughing or
(ED), accounting for around 2% of all atten- vomiting. This causes raised pressure
dances.1 2 within the superior vena cava (SVC),
In this article, we discuss the aetiology and with consequent pinpoint petechiae in the
an initial assessment of NBR in children. distribution of the SVC alone (above the
Aetiology nipple line).1
The most common causes of NBR in chil- However, the early stages of serious
dren can be broadly classified as infective or bacterial infections may present with a
mechanical. Other causes are less common localised NBR meaning that a mechanical
and are classified as vasculitic, haematolog- cause is often arrived at through a process
ical and ‘other’1–6 (table 1). of exclusion.
Direct trauma can result in bruising that
Infectious causes
can appear identical to a true NBR. There
Any serious bacterial infection (SBI) can
is usually a clear history of trauma. In
result in a NBR via disseminated intravas-
cases where a traumatic cause is likely, it is
cular coagulation (DIC). Some infections,
important to consider safeguarding. This is
however, feature a NBR as an early sign.
especially important when lesions are local-
The most common infections associated
ised to the genital area, buttocks, are unusual
with a NBR as an earlier sign are as follows.
or linear, or when the history is unclear.7
►► Viral:
To cite: Waterfield T, –– Enterovirus and adenovirus are the
Dyer EM, Lyttle MD. Arch
most common infectious causes of Vasculitic causes
Dis Child Educ Pract Ed Henoch-Schonlein purpura (HSP) is the
Epub ahead of print: NBR in children.3
[please include Day Month ►► Bacterial most common vasculitic cause in children,
Year]. doi:10.1136/ –– Streptococcal infections
1–4
with other less common causes including
archdischild-2017-313998 –– Meningococcal disease (MD).
6
atypical Kawasaki disease, polyarteritis
Waterfield T, et al. Arch Dis Child Educ Pract Ed 2018;0:1–5. doi:10.1136/archdischild-2017-313998 1
Copyright Article author (or their employer) 2018. Produced by BMJ Publishing Group Ltd under licence.
Downloaded from http://ep.bmj.com/ on March 8, 2018 - Published by group.bmj.com
Best practice
Figure 1 Petechiae and purpura typical of invasive meningococcal disease. Petechiae are non-blanching spots that are <2 mm in size and are
due to capillary haemorrhage. As more haemorrhages occurs the petechiae coalesce into purpura (>2 mm). Images used with permission of the
Meningitis Research Foundation.
Table 1 Causes of non-blanching rashes in children with common features and possible investigations
Cause Features Investigations
Infective Fever may or may not be present—look for Full blood count
worrying features: C-reactive protein
►► Appearing unwell Meningococcal PCR
►► Irritable/lethargic Viral PCR
►► Prolonged capillary refill time Blood culture
►► Spread of rash
Blood gas
►► Purpura
Glucose
►► Deterioration
Haematological Isolated thrombocytopenia in a well child Full blood count
►► Idiopathic thrombocytopenia purpura (ITP) Abnormal film or cell count (not ITP) Blood film
►► Malignancy Deranged clotting in a well child Coagulation studies
►► Coagulopathy
Mechanical Identification of mechanical cause in an Not always needed—when performed they are
otherwise well child. normal.
Henoch-Schonlein purpura Well child, classical rash, no spread or Urinalysis for evidence of glomerulonephritis
deterioration. Normal cell counts and film. Blood pressure
Where unclear may need to rule out other
serious causes.
Best practice
Other causes children with fever and NBR will have SBI, it is also
It is worth considering whether a well-child’s rash is important to note that not all children with SBI and
in fact a normal variant. A study of infants attending NBR present with a fever—up to 20% of cases of MD
routine health checks found that petechiae were have no fever at presentation to ED.1 18 While it may
commonly identified in well infants with over be reasonable to withhold antibiotics from children
one-fourth having one or more petechiae.17 who appear well, it is important to note that children
can initially appear well and deteriorate, mandating a
Assessing the child period of active observation.
No guideline or algorithm will ever perform perfectly due
to the range of possible causes. The approach discussed
Can I make a positive diagnosis?
here is designed to assist the thought process but is not a
If the child appears well, it is still important to attempt
substitute for clinical reasoning or experience.
to make a diagnosis. While undertaking this process, it
The steps involved are outlined in figure 2 and
is important to carefully monitor the child for signs of
include:
deterioration. As even well-appearing children may be
►► An initial assessment of wellness to identify those
requiring immediate treatment.
harbouring an occult SBI.
►► An attempt to make a positive diagnosis. When searching for a diagnosis, a number of tests can
►► If no positive diagnosis can be made then consider if it is be considered (table 1). National Institute for Health
appropriate to discharge. and Care Excellence (NICE) meningitis (bacterial) and
meningococcal septicaemia in under 16 s: recogni-
Is the child well? tion, diagnosis and management guidance includes the
Any child who appears unwell with a NBR should be assessment of NBR in febrile children and advises.19
presumed to have a SBI and be treated accordingly.1–5 ►► FBC;
In the context of NBR, presenting features of irrita- ►► C-r eactive p rotein(CRP) ;
bility, lethargy or a prolonged capillary refill time ►► c oagulation screen;
confer a significantly increased risk of SBI1–5—children ►► b lood culture;
with these features should be treated as per national ►► w hole-blood PCR for Neisseria meningitidis ;
guidance immediately. ►► b lood glucose;
►► Meningococcal disease (meningitis (bacterial) and ►► b lood gas.
meningococcal septicaemia in under 16 s: recognition, These investigations focus on the diagnosis of MD.
diagnosis and management). While this is important, table 1 below outlines some
►► Sepsis: recognition, diagnosis and early management. additional investigations and anticipated results for
The presence of fever is a key component of the other causes of a NBR:
history and examination, though it should not be relied Special considerations are required for children
on in isolation to make clinical decisions. While not all presenting with purpura. These children are at higher
Best practice
risk of MD and other SBI than those with petechiae a child, it is important to provide clear advice to return if
alone, and some therefore advocate that all chil- there is any: deterioration in the child’s health, spread of
dren with purpura should be treated for suspected the or change of the rash.
MD/sepsis.1–5 This approach, while safe, leads to The current best evidence for the management of this
overtreatment of children with HSP.5 There are no group comes from the Newcastle-Birmingham-Liverpool
unifying diagnostic criteria for HSP, but the presence algorithm (NBL).5 This algorithm has been validated
of palpable purpura in a characteristic distribution in with a reported sensitivity of 100% and a specificity of
an otherwise well child suggests HSP as opposed to 82% for the diagnosis of MD.5 In the NBL algorithm, a
MD.5 However, it is known that even experienced child can be discharged if the child remains well, has no
paediatricians may misdiagnose MD as HSP, leading purpura, no spread of the rash over 4–6 hours of observa-
to treatment delays.5 tion and a CRP <6 and white cell count 5–15 10^9/L .5
A list of common causes and possible investigations This approach outperformed current NICE guidance in
are outlined below in table 1. a comparative validation exercise, with NICE guidance
displaying a sensitivity of 97% and specificity of 50%.5
Can I rule out serious illness? The difference in the performance of the two algorithms
If a positive diagnosis cannot be made but the child was statistically significant (P<0.001).5 Both NICE and
otherwise appears well, then the more difficult ques- NBL algorithms are designed as ‘rule-out’ algorithms
tion is ‘Can I rule out SBI and other serious causes?’ and as such both are highly sensitive but poorly specific.
Proving a negative is always more difficult in medicine This means that very few cases of MD will be missed but
and this is where the real challenge in managing child- that many children will receive unnecessary treatment.
hood NBR occurs, given that so few have a serious under-
lying cause. Where determining a cause is not possible, Summary
the challenge is deciding who is safe to be discharged and NBRs are a common reason for children presenting to
who should be treated3? If a decision is taken to discharge healthcare, often with non-specific findings. While SBI
Best practice
is rare, it is important to promptly identify and treat 11 Provan D, Stasi R, Newland AC, et al. International
those at greatest risk. For well-appearing children, a consensus report on the investigation and management of
structured approach can lead to a positive diagnosis primary immune thrombocytopenia. Blood
in many, coupled with safe discharge decision-making. 2010;115:168–86.
12 Tilden W, Valliani S. Severe thrombocytopenia and recurrent
Contributors TW conceived the idea and wrote the majority of the article. epistaxis associated with primary Epstein-Barr virus infection.
ED contributed to sections on haematological causes, vasculitic causes and BMJ Case Rep 2015;2015:bcr2014208018.
mechanical causes. ML provided a comprehensive review of the article and 13 Chaudhry R, Wegner R, Zaki JF, et al. Incidence and
expert analysis. All authors agreed the final version.
outcomes of heparin-induced thrombocytopenia in patients
Competing interests None declared.
undergoing vascular surgery. J Cardiothorac Vasc Anesth
Provenance and peer review Commissioned; externally peer reviewed. 2017;31:1751–7.
© Article author(s) (or their employer(s) unless otherwise stated in the text of 14 Urbonas V, Eidukaitė A, Tamulienė I. The predictive value of
the article) 2018. All rights reserved. No commercial use is permitted unless soluble biomarkers (CD14 subtype, interleukin-2 receptor,
otherwise expressly granted.
human leucocyte antigen-G) and procalcitonin in the detection
of bacteremia and sepsis in pediatric oncology patients
References
with chemotherapy-induced febrile neutropenia. Cytokine
1 Wells LC, Smith JC, Weston VC, et al. The child with a non-
2013;62:34–7.
blanching rash: how likely is meningococcal disease? Arch Dis
15 Moulis G, Sommet A, Sailler L, et al. Drug-induced immune
Child 2001;85:218–22.
thrombocytopenia: a descriptive survey in the French
2 Mandl KD, Stack AM, Fleisher GR. Incidence of bacteremia
pharmacovigilance database. Platelets
in infants and children with fever and petechiae. J Pediatr
1997;131:398–404. 2012;23:490–4.
3 Nielsen HE, Andersen EA, Andersen J, et al. Diagnostic 16 Clarke RT, Van den Bruel A, Bankhead C, et al. Clinical
assessment of haemorrhagic rash and fever. Arch Dis Child presentation of childhood leukaemia: a systematic review and
2001;85:160–5. meta-analysis. Arch Dis Child 2016;101:894–901.
4 Brogan PA, Raffles A. The management of fever and petechiae: 17 Downes AJ, Crossland DS, Mellon AF. Prevalence and
making sense of rash decisions. Arch Dis Child 2000;83:506–7. distribution of petechiae in well babies. Arch Dis Child
5 Riordan FA, Jones L, Clark J. Non-Blanching Rash Audit 2002;86:291–2.
Group. Validation of two algorithms for managing children 18 Hart CA, Thomson AP. Meningococcal disease and its
with a non-blanching rash. Arch Dis Child 2016;101:709–13. management in children. BMJ 2006;333:685–90.
6 Bourke TW, McKenna JP, Coyle PV, et al. Diagnostic accuracy 19 NICE. Meningitis (bacterial) and meningococcal septicaemia
of loop-mediated isothermal amplification as a near-patient test in under 16s: recognition, diagnosis and management |
for meningococcal disease in children: an observational cohort Guidance and guidelines | NICE. 2015 https://www.nice.org.
study. Lancet Infect Dis 2015;15:552–8. uk/guidance/cg102 (cited 2017 Oct 10).
7 Burrows NP. Purpura in infants and children. J Am Acad
Dermatol 1998;39:661–2.
8 Bangia AV, Kamath N, Mohan V. Ranitidine-induced Answers
thrombocytopenia: a rare drug reaction. Indian J Pharmacol
2011;43:76. A. C Discuss with haematology
9 Barut K, Şahin S, Adroviç A, et al. Diagnostic approach and B. E Fluid bolus and intravenous antibiotics
current treatment options in childhood vasculitis. Turk Pediatri C. B Henoch-Schonlein purpura
Ars 2015;50:194–205. D. B Discharge with safety netting advice
10 Buchanan GR. Immune thrombocytopenia during childhood: E. C Top to toe examination and consider safeguarding
new approaches to classification and management. J Pediatr background checks
2014;165:437–9.
These include:
References This article cites 18 articles, 9 of which you can access for free at:
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Notes