Showing posts with label Non-blanching rash. Show all posts
Showing posts with label Non-blanching rash. Show all posts

Tuesday, 30 April 2019

Rashes in children: What is the diagnosis? - Probably a virus

A rash is a very common feature of a paediatric presentation and is often the primary reason for seeking medical advice.

I think that clinicians also sometimes feel a bit of anxiety about rashes.  What does the rash mean?  Should I be able to diagnose the illness based on the rash?

The rash can be diagnostic but often it is not.  Even when the rash gives a specific diagnosis, that diagnosis is usually a virus and treatment remains symptomatic and supportive.

These are some important questions to have answers to when assessing a child with a rash.
  • Is the child well? If not, how unwell are we talking about and for how long?
  • Is the child febrile?
  • How did the rash start?
  • How has the rash changed since it started?
  • Is the rash itchy?
When examining the child, it is important to avoid the temptation to focus excessively on the rash.  The child should have a systemic examination that will identify any cardio-respiratory, abdominal or neurological abnormality.

The rash itself is then in context of an assessment that has determined whether the child is significantly unwell or has any significant abnormal findings.  What this tells us is whether the rash is of importance because the child is quite unwell, or more of interest since the child is well.  In the well child with no significant abnormal findings, a diagnostic rash can still give useful information in terms of prognostication and the ability to give specific advice about what to do from a infection control point of view.

Let’s look at some specific rash related diagnoses that are accompanied by non-specific symptoms such as pyrexia-

Roseola Infantum
What does it look like?
This infection is most commonly seen between the ages of 6 months and 3 years.  The classical presentation is of a significant fever but a surprisingly well child with non-specific symptoms such as coryza and pharyngitis.  Essentially, the child has all the signs of a viral upper respiratory tract infection (URTI) but with an impressive fever.

A macular patchy erythematous rash often appears as the fever starts to resolve.  Typically the rash is more prominent on the trunk than limbs.
What causes it?
Human herpes virus 6

What specific advice is there for this diagnosis?
None.  Treatment is symptomatic.

Chickenpox
What does it look like?
Chickenpox is a vesicular (small fluid filled lesions) rash which is usually found all over the body.  Children are usually either mildly febrile and unwell in the first few days, or not unwell at all.  The rash is often itchy.
What causes it?
Varicella zoster virus

What specific advice is there for this diagnosis? 
There is no specific treatment.  If the child seems unwell then paracetamol (acetaminophen) is the preferred treatment for systemic symptoms.  While there is some concern about using ibuprofen, the evidence strongly suggests that this concern is unfounded.  However, most children do not become significantly unwell with chickenpox and it is unusual for symptoms to require more than paracetamol.  If a child with chickenpox is very unwell, that is a clinical situation that mandates a careful assessment to consider the possibility of sepsis, usually in the form of invasive streptococcal infection.
Itching can be treated with antihistamines.  In the UK, it is usual practice to ask that the child is kept out of school or nursery until day 7 of the rash, at which point new lesions are not forming and the existing spots are crusting.

Hand, foot and mouth disease
What does it look like?
Vesicles on the face around the mouth, ulcers inside the mouth, vesicles on the hands and feet and perianal vesicles or ulceration.  (Somehow the perianal bit got left out when naming this childhood infection)  The child is usually systemically well but when the rash is appearing may be a little miserable and pyrexial.

What causes it?
Coxackie virus

What specific advice is there for this diagnosis?
Treatment is symptomatic.  This is a good opportunity to practice the philosophy of “treat the child, not the fever.  Many children with hand, foot and mouth disease are not febrile but may be in significant discomfort from the oral lesions.  The importance of analgesia to help the child be comfortable enough to drink should be emphasised.
The UK public health advice for hand, foot and mouth disease is that in itself, it does not mandate and absence from school or nursery.

Pityriasis rosea
What does it look like?
The classical pityriasis rosea rash starts with a herald patch in the form of a well localised erythematous area somewhere, usually on the trunk.  This may go unnoticed and if seen rarely causes alarm.  The generalised rash that follows is what usually leads to the seeking of a medical opinion.  This rash is an impressive patchy pink rash with the pattern of the patches following the lines of the dermatomes of the skin on the trunk, forming what is described as a “Christmas tree” distribution.

What causes it?
Human herpes virus

What specific advice is there for this diagnosis?
The child is usually well at the time of the Christmas tree-like rash appearing so no specific treatment is needed.  It should be explained that the rash may last for a few weeks.  There is no need for the child to be excluded from school or nursery.

Slapped Cheek Syndrome (Fifth Disease)
What does it look like?
Most of the features are non-specific: Fever, coryza, sore throat.  The name comes from the typical bright red rash which appears (usually) on both cheeks.  The redness is both more impressive and more consistent than the flushed cheeks seen in febrile children.  This is often followed by a more non-specific, patchy, popular, blanching erythematous rash on the rest of the body.

What causes it?
Parvovirus

What specific advice is there for this diagnosis?
For the purposes of managing the child, treatment is symptomatic.  Most cases of slapped cheek resolve without complications.

A rare but significant complication of parvovirus is an aplastic crisis secondary to the effect of the virus on the bone marrow.   A history of recent parvovirus infection followed shortly afterwards by significant or atypical illness or pallor should prompt the testing of a full blood count.

Parvovirus infection in pregnancy carries a risk of miscarriage or hydrops fetalis (due to the same aplastic crisis).  Parvovirus is not treatable and most pregnant women are immune.  In most places, the advice for pregnant women who come in contact with parvovirus is to seek medical assessment if they subsequently become ill, especially if they develop a rash of any kind.  If serology confirms parvovirus infection then the pregnant woman should be referred to the fetomaternal team.  Through the marvels of modern medicine, it is now possible to transfuse a baby in utero and potentially keep them well long enough to reach a gestation where it delivery is an option.

Measles 
What does it look like?
Typically the child is febrile, coryzal and coughing for a couple of days before the rash appears.  The rash itself is an erthematous maculopapular rash which usually starts on the head before spreading to the rest of the body.
Kopliks spots are diagnostic but rarely seen as they don't hang around for long.  These are small white spots that appear on the inside of the cheeks, opposite the molars.
Measles should be suspected when a child has significant non-purulent conjunctivitis or is particularly miserable despite analgesia.  Unlike uncomplicated viral illnesses, the child is usually quite unwell several days into the illness when the rash appears. (Consider a differential diagnosis of Kawasaki Disease in the child who has had fever for five days as many of the features overlap.  Unlike Measles, early specific treatment for Kawasaki Disease is essential)

What causes it?
Measles virus

What specific advice is there for this diagnosis?
Measles infection requires specific infection control measures and in the UK is a notifiable disease.  There is no specific treatment for Measles and at presentation, the key decision is about how unwell the patient is.  If well enough to be managed at home, it is very important to avoid unnecessary admission to hospital as this might lead to infection of those most at risk.  However if the child is showing signs of significant infection (mainly encephalitis) then admission is probably necessary.  If referring to secondary care it is essential that the accepting team are aware that Measles is suspected so that the child can be kept away from others from the moment of arrival to hospital.

Viral Urticaria
What does it look like?
It looks like an allergic reaction or nettle sting.  The itchy, raised red and white rash can be seen in any part of the body and can be quite alarming.  Typically this rash appears as the illness is getting better.  Lesions appear and disappear several times a day.  If the onset of the rash is accompanied by other symptoms appearing (such as wheeze, oral swelling or vomiting) then acute IgE mediated allergy should be suspected.  Viral urticarial should not be accompanied by the appearance of these symptoms.

What causes it?
One of many possible viruses

What specific advice is there for this diagnosis?
It should be explained that the rash is caused by the virus and the child’s immune system.  The rash doesn’t tell us anything specific about the infection and doesn’t mean anything bad about the illness or the child.   While anthistamines may reduce the itching, they do not seem to make the rash go away any faster.  The rash will usually resolve spontaneously over the space of several days.

It is notable that children who develop viral urticaria are sometimes taking antibiotics when the rash appears.  This can cause concern regarding possible drug allergy.  The evidence suggests that a large number of children developing rashes while taking antibiotics are simply manifesting a viral rash (including urticaria).  This association is contributing to the overdiagnosis of antibiotic allergy.  Many specialists are now advising that a label of antibiotic allergy is not given to a child if they have an acute illness that could be viral, the only symptom is a rash and it is the first time the child has had a rash while taking antibiotics.

Non-specific Viral Rash
What does it look like?
These rashes can appear during the acute infection or recovery phase of the illness.  Typically the rash is a diffuse, patchy erythema.  It may be macular or papular.  In the majority of cases, all of the rash blanches.  Occasionally, a few petechiae can be found.  In a population vaccinated against most strains of meningococcus, a small number of petechiae is most likely to be part of a viral rash.  Indeed, finding one or two petechiae is within normal for a well child at any point. (1)
What causes it?
Any virus that is on the rash B-team could be responsible.  If it's not a diagnostic rash, you can't make a specific diagnosis.

What specific advice is there for this diagnosis?
It's important to explain that the rash doesn't have any specific meaning. For example, a child with this rash does not need to be kept out of school, for infection control reasons at least. Safety-netting advice should mainly centre around the illness, not the rash.  The rash may well persist after the child's illness has resolved.

Many specific rashes start of as non-specific so if the rash changes significantly they may need to be reassessed.  In particular they should know how to assess for non-blanching rash.

Erythema Multiforme
What does it look like?
As the name (What, no Latin?) suggests, it is a rash with multiple forms.  The rash varies from place to place rather than being uniform in appearance.  The rash varies in appearance and texture.  The typical target lesions that also help make the diagnosis are circular and have a dark red centre.
What causes it?
The rash is in many ways very similar to urticaria in children.  It may be a drug reaction but is more commonly triggered by a virus.  That virus is not usually specifically identified.  Atypical bacterial infection, most commonly mycoplasma, may also trigger erythema multiforme.

What specific advice is there for this diagnosis?
Essentially the same applies to Erythema Multiforme as applies to viral urticaria and non-specific viral rashes.  If there is no obvious specific cause, safety-netting for the illness is most important.

Because Erythema Multiforme can rarely progress to Stevens-Johnson Syndrome, it is worth advising the family to seek reassessment if the child develops an inflamed mouth.

Henoch-Schonlein Purpura (HSP)
What does it look like?The typical HSP rash is a purpuric rash on the lower limbs, predominantly on the buttocks and extensor surfaces.  This is often fully apparent at presentation but sometimes the initial rash is not purpuric.  In some cases other symptoms precede the rash.
Typical symptoms of HSP include leg pains and abdominal pains, though in some cases HSP is asymptomatic.

What causes it?
The cause is unknown, however it is presumed that this vasculitic process is triggered by infection.  In that sense, it can be considered a viral rash.


What specific advice is there for this diagnosis?
Most cases of HSP are suitable for outpatient management and in many cases this is well within the remit of the General Practitioner.  A full explanation of the condition, possible complications and how to manage/ followup can be found here.

Bringing it all together
So there you have it - a reasonably comprehensive list of common rashes seen in childhood infections.  In most cases, the rash will not give a specific cause.  In every case, the clinical condition of the child is by far the more important part of the assessment.  After all, it's probably a virus and you probably can't treat that.

Edward Snelson
Rash decision maker
@sailordoctor

Disclaimer - it might not be.
References
  1. Downes AJ, Crossland DS, Mellon AF Prevalence and distribution of petechiae in well babies Archives of Disease in Childhood 2002;86:291-292.

Wednesday, 12 April 2017

Henoch Shonlein Purpura - Who, What, Where and When?

In Paediatric Emergency Medicine in the UK, it is often the case that a referral requires me to choose between more than one specialty.  For example cuts on the face (the ones that require suturing under general anaesthetic) could be referred to either plastic surgeons or maxillofacial surgeons.  Hand fractures are sometimes looked after by the orhopaedic surgeons (note use of their Sunday name) and sometimes by plastics.  Now that I think about it, the overlap almost always involves plastic surgery.

The decision about who to refer a patient to will depend on various factors.  These include resources, availability and the particulars of the case.  Matching the child and the illness (or injury) to the right person to follow the case up is part of the art of Primary Care.  After much thought and consideration, I've decided to refer children with Henoch Schonlein Purpura (HSP) to the very best.  I refer them to their General Practitioner.  
(Ankle bracelet not included.  See in store for more details)

The reason for this is that General Practice does two things better than any other specialty.  General Practice excels at avoiding the harm done by unnecessary tests and treatments, and (in my opinion) no other specialty in medicine is so good at providing the continuity needed to monitor a condition.  This allows the patient to receive appropriate care while at the same time having an experienced clinician involved who will intervene if needed.  For uncomplicated HSP, that is exactly what is needed.

HSP is the most common vasculitis in children.  Nobody really knows why children get it but the number one suspect is of course 'recent infection'.  These are always getting the blame for mysterious childhood illnesses.  If you ask me, I think it is institutional infectionism.

HSP is best known for causing a purpuric rash on the buttocks and lower limbs,  While this is the typical rash, atypical rashes are pretty common.  The thing that is most consistent about the HSP rash is the following triad:

HSP is a clinical diagnosis.  It is usually identifiable through a typical presentation and because other possible causes of non-blanching rash can be ruled out clinically.  Sepsis, leukaemia and immune thrombocytopaenia are some of the possibilities that should be considered if the presentation is not that of typical HSP, or if the child is unwell, or anaemic or has splenomegally (etc. etc.).

It is also worth mentioning that not all children have the courtesy to present with their symptoms in the correct order.  Everyone know that the rash should come first.  Some children choose to ignore this and complain of aches and pains before the rash appears.  Also, before the rash becomes purpuric it can start out as non-specific erythema or even look a little urticarial.

I am often asked if blood tests are needed for a child when diagnosing HSP.  My answer is this:  
For example, I don't do CRPs to diagnose sepsis.  I use a CRP once I have diagnosed sepsis because that will help in the ongoing management.  In injury, I use X-rays where it is probable that the X-ray will alter my management.  I don't use CXR to diagnose pneumonia.  I use CXR if I suspect complicated or atypical pneumonia.  In HSP diagnosis, tests are really for uncertainty in those cases where the presentation is ambiguous or atypical.  If the presentation is unambiguously HSP, no diagnostic tests are needed.  There are tests to be done but those are screening for complications.

Most cases of HSP follow a benign course.  The rash persists for several weeks and there many children experience aches and pains in their legs and abdomen.  However, symptoms other than the rash will typically settle in the first week or couple of weeks.  Things don't always go this way though.  There are four possible ways for the course of HSP to be problematic.

Problem 1) Extreme symptoms.

Pain is a funny thing and in some cases of HSP, despite a lack of significant complications, some children and young people get severe pain (despite good doses of simple analgesia) in their legs or abdomen.  These children should be referred acutely to be seen by a paediatrician.  This is both to enable management of the pain and to look for more significant complications.

Problem 2) Non-renal complications of HSP

I've said that leg and abdominal pains are fairly common symptoms of HSP.  It is also true that everything in paediatrics has an evil twin.  In the case of leg pains, it is possible for HSP to cause a significant polyarthritis.  If a child has swollen joints, the pain is severe or they develop a significant limp - refer.

Similarly, if a child with HSP shows signs of severe abdominal pain, consider the possibility that they have developed one of the most significant complications of HSP - intussusception.  Other signs might include pale episodes and blood or 'redcurrant jelly' in the stools.  With or without these other signs, assessment by a paediatric surgeon is urgently needed if abdominal pain becomes acutely severe.

Almost anything that can go wrong can go wrong in HSP.  Involvement of other organs than kidneys, skin, bowel and joints is rare.  Atypical symptoms such as headache or chest pain should be taken very seriously and the child should be referred acutely.

At some point, all of these factors will lead to a decision about where the child is managed.  I think that the decision usually looks like this:
There is nothing mandatory about referring a clear cut case of HSP at first presentation.  However, most GPs that I know would prefer an initial assessment in secondary care, even if the child is extremely well and has no symptoms with their non-blanching rash.

Problem 3) Renal complications

While aches and rashes are what cause children and parents to lose sleep over HSP, it is the possibility of renal complications that usually worries the clinician.   A significant chunk of children who have HSP will develop a mild nephritis.  A smaller number go onto get a significant nephritis and a small number develop severe, kidney-threatening nephropathy.  Don't worry too much about the numbers as they don't really help in a case series of one.  What is true is that it is very likely that the child in front of you will either never develop a renal problem, and if urine dip flags something up, it is most likely to be a transient mild haematuria or proteinuria.  In order to identify those cases of silent but significant nephritis, monitoring is needed for all cases of HSP.

Exactly how much monitoring is needed is slightly unclear.  Advice is based on clinical experience, common sense and a bit of logic.  If you put those things together, you might come up with this:
This is followup strategy is based on the well child, who has no significant haematuria or proteinuria at any point.

What this aims to do is detect renal involvement early, while avoiding any OCD type behaviour (such as daily urine dipsticks).  The length of follow-up is debatable but is based on a cohort study that showed that no child with HSP developed renal involvement after 6 months.  Roughly half the guidelines from big centres recommend follow up to 6 months and half say 12 months.

There are those that advocate longer followup (including lifelong annual reviews) for all cases.  However, I suspect that there is a significant effect of bias driving that.  If a child has had a complicated case of HSP, this may well be warranted, but if the course of the illness was benign I doubt that they will develop late complications.  The Nottingham renal unit recommends lifelong monitoring if everything else has been normal but there was at least one occasion where the urine dip showed ++ or more of protein. (1) This stratification allows for the possibility of nephropathy in selected cases rather than ongoing screening for all.

The need for a blood pressure check after the first couple of visits is also debatable (thus the *).  By continuing to check BP after it has been normal on two occasions, are we saying that the child could have had renal involvement severe enough to cause hypertension, yet this never showed up as a positive dipstick?  This seems very unlikely to me and I have never found a paediatrician who has seen such a thing happen.  Despite this, all guidelines and review articles that I have seen recommend that BP is checked at every visit.

Problem 4 - Prolonged or relapsing course of illness

This brings me neatly onto the final thing that might cause a clinician to feel that the child needs more investigation and management than can be given in Primary Care.  In some cases, the course of HSP does not follow the typical one of a few weeks of rash and a few days of other symptoms.  The symptoms might continue for months, or relapse after initial remission.  A mild relapse is actually quite a common occurrence in HSP and worth warning the family about.  If a relapse is severe or prolonged I would suggest that these children also need to be referred.  The paediatrician will need to review the diagnosis (in case the reason for an atypical course is that the diagnosis is wrong) and consider whether to investigate e.g. for an autoimmune cause of the rash.

HSP FAQs

Q. Does the child need blood tests at presentation?

A. Only if there is diagnostic ambiguity or if the urine dip/ BP is abnormal.


Q. Do steroids prevent complications?

A. No, but they may have a role in treating complications of HSP. (2) Other interventions that have found to be unhelpful include antiplatelet treatment or immunosupression.


Q. What counts as hypertension in a child?

A. This is difficult question to give a straight answer to.  It's not a lack of people coming up with definitions that is the issue.  It's more that we don't know much about what levels of hypertension cause what effect in the long term.  Most guidelines go for something like systolic BP >95th centile on three occasions.  It is important to use the correct size of BP cuff for the size of the child and to use an appropriate reference range. (3)


Q. So should I be checking BP at every visit even if the urine has been normal (no more than a trace of blood and no more than + protein) on every test?

A. If you didn't, you would be going against all the guidelines in existence.  I still haven't had a good explanation as to why it is really necessary, but nor have I convinced others that it is unnecessary, so for now you should keep on doing it.


So in summary, managing HSP is relatively straightforward.  After initial assessment, most can be monitored by their GP.  Some will need to stay under the care of a paediatrician and a small number will need to be referred on, usually to a nephrologist.  For the uncomplicated cases, there is no absolute certainty about often or how long to screen for renal involvement, so guidelines have to be mostly based on pragmatic expert opinion.

So, who, what, where and when?  Me, you and maybe them doing as much nothing as possible until we are sure that everything is OK.  Simple really.

Edward Snelson
Dogmatologist
@sailordoctor


I would like to express my deepest gratitude to the young person who consented to have photos of her HSP rash used for this purpose.  Thank you.


References
  1. Henoch-Schönlein Purpura (HSP) Guideline, Nottingham Children's Hospital
  2. W Chartapisak et al., Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review, ADC, Feb 2009
  3. Blood Pressure Levels for Boys by Age and Height Percentile, National Heart, Lung and Blood Institute