What can you learn from rashes?

CPD
What can you learn from rashes?
An approach for children.
Marh F Cotton M. Med (Paed),FCPaed (SA), DTMAH, DCH (SA)
Helena Rabie FCPaed (SA), M. Med (Paed)
H Simon Schaaf M. Med (Paed),DCM, MD (Paed)
HJ Jordaanx M.Med (Derm)
Paediatric Infectious DiseasesUnit, Dept. PaediatricsA Child Health, Ty*gerbergChildren's Hospital,
Department of Dermatology
Correspondence to: Stellenbosch University Faculty ofHealth Sciences,Tygerberg, 75o5.
Dr Mark Cotton: , Fax - ozr 938 9138, Tel - ozr 938 grzT
Introduction
Rashescausea great deal of concernto parentsand are often dismissedas either inconsequentialor confusing by
health care practitioners.While the majority of rasheshave trivial consequences,
othersare manifestationsof
seriousand potentially fatal disease.A careful evaluation of a rash can be extremely rewarding. Seriousdiseases
can be diagnosedwith a reasonabledegreeof certainty and appropriatetherapy rapidly instituted. For example,
Kawasaki diseaseoften presentswith fever and a rash and failure to recognize it in time and give intravenous
gammaglobulin will result in coronary artery aneurysmsin 20Yoof patients. In contrast, parents can often be
reassuredof the benignnatureof many rashes. (SAFam Pract zoog;45(ro): z8-g4)
Specificfeaturesof rashes
A good clinical history and physical
examinationare important for accurate
diagnosis.Important points in the history
are presentedin table I. An important
and often neglectedfeature is the tempo
of development.For example,the petechiaeseenin meningococcaemiadevelop over minutes to hours, while in
papulonecrotictuberculid @NT), a form
of cutaneoustuberculosis, the lesions
developover weeks.
Importantpoints in physical examination are shown in table II. Kawasaki
diseaseis diagnosedby a constellation
ofphysical signs such as maculopapular
rash,bulbar conjunctivitis,swollen digits and unilateral cervical adenopathy.
Occasionally severe diseasesuch as
ecthymagangrenosummight be missed
if the nappy is not removed.
Type ofrash (Table III)
There are many types of rashes,including maculopapular,erythrodermal,vesicularor bullous, petechialor purpuric
z8
and erythema nodosum. The majority
of rashesare maculopapular.Somerashes may begin as either macular or maculopapular and evolve over time. For
example, both meningococcal disease
and Henoch-Schonleinpurpura may
begin as macules/papulesthat become
purpuric over one or two days.
Probably the most serious viral
causeof a maculopapularrash is measles.The rashis accompaniedbycoryza,
coughing and conjunctivitis. Koplik's
spots are pathognomonic of measles
and should always be sought. They are
seenon the buccal mucosal a day preceding and two days into the rash. Measles spreadsvery rapidly and immunocompromisedpatients are extremely
vulnerable.Failure to recognizemeasles,
especiallyin the hospital environment,
and institutepost-exposureprophylaxis
to unimmunized contacts (vaccine in
immunocompetentcontact and immunoglobulinsin severelyimmunocompromised)will have seriousconsequences.Due to successfulmassvaccination
programs, measlesis now rare and may
not be recognizedbymany clinicians.
Also, the maculopapular rash may be
harder to identiff in patientswith dark
skin. (Figure 1A and B)
Figure 1. A) 18 year old girl with
measles.Notethe dischargefmm eyes
and nose.
Figure 1. B). Maculopapularrashon
dark skin
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Table 1. Questionsto consider in a child with a rash
Question
Examples
Possible diagnosis
Characteristic features
How old is the oatient?
Neonates
Congenital cytomegalovirus, rubella or
toxoplasmosis @lueberry muffin syndrome)
Erythema toxicum
Discrete, palpable purpuric lesions
Congenital syphilis
Wheredid the rashappearfirst?
What is the tempo of development?
Any unusual exposure
Recent contact with an
ill person? (incubation
period)
First year of life
Roseola infantum
Behind the ears, spreading
downwards
Face fint, spreading down
Measles
Minutesto hours
Meningococcaemia
Hoursto days
Days
Daysto weeks
Water
Drug and viral
Infective endocarditis
Infective endocarditis,collagen-vascular,
malisnancv. tuberculosis
Schistosomiasis
Rodents
Livestock
Leptospirosis
Brucella
Unpastewised milk or
Listeriosis
Rubella
Maculopapular with yellow centre.Appears within
days ofbirth
Vesicular or maculopapular. Involve palms and
soles,often desquamating
Maculopapular rash occurs at defervescence after
3 to 4 davs ofhish fever
frocrome oI J-4 oays: Iever, conJuncunns, coryza,
cough and Koplik spots. Rash spreadsover 2-3 days.
Fever for one day. Occipital lymph nodes palpable.
Distincl macu
rash
Maculopapular or petechial rash which may evolve
to purpura andlor ecchymosis
Usually maculopapular
"Swimmer's itch" - pruritic papular rash within 24
hours ofexoosure
Rare - Maculopapular rash
Rare - Maculopapular, erythema nodosurn,petechiae,
vasculitic lesions
Exposure to the mother - maculopapular rash part
7 days
Measles
Chicken pox, rubella
7 - l0 days
Table II. Special features of the rash on examination
Comments
Distribution
Hands and feet involved
Most prominenton legs
Inguinal area
Rickettsial infection
Enterovirus
Syphilis
purpwa
Henoch-Schdnlein
Ecthyma gangrenosumdue to
Rubella
Scarlet fever
Parvovirus
Vesicular
Chickenpox
Coxsackie (Hand-foot-&-mouth disease)
Toxic epidermal necrolysis
"Sunbum" like rash
Discharge absent
Kawasaki disease
Stevens-Johnsonsyndrome
Eschar(often abovehairline)
Vesicles in posterior oropharynx
Palpablepurpwic lesions(begin aserythematouspapules)
Initiate antipseudomonaltreatment.The diagnosis may
be missedifthe doctor doesnot removethe
Uough,coryza,conJunchvltrs.
Kopl*'s spots
Red eyes, stomatitis, strawberry tongue, swollen hands
and feet (digits are warm), cervical lymphadenopathy
Usually associatedwith leukocytosis,raisedC-reactive
protein and erythrocyte sedimentationrate
Occipital lymphadenopathy
Intenselyerythematous
"Slapped cheek" appearance.Rash has "latticelime"
reticularpattemwith centralclearing
All stagesseenat once:papules,vesiclesand crusts
Mucosal and eye involvement. May be drug-related
Patientshocked.multi-orpand function
promrnent rn
spotson
buccalmucosafor measles
Look for swollen digits (warm on palpation [as opposed
1oconditionsassociatedwith generalizedoedemaand
whereperipheriesare cooler]).
Mucositis andurethritismay be present.
Swollen hands and feet
Strawberrytongue
Hard
buccal mucosa
Streptococcttspyogenes(includes scarlet fever)
Toxic shock syndrome
gococcaemia,Papulonecrotictuberculid,
Histiocytosis, T cell lymphoma
Ecthyma gangrenosum
Hepatosplenomegalymay accompany
Lesionsblack - most often dueto
Pseudomonasaeruginosa
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Table III. Differential diagnosis of rash with a fever (adaptedfrom Nelson Textbook of Pediatrics,l4th edition)
Type ofrash
Differential diasnosis
Macular or maculopapular
Viral: measles,rubella, roseola infantum, enteroviruses,parvovirus Bl9 (slapped cheek disease),Ebstein-Ban virus
(infectious mononucleosis),exanthem subitum (HSV 6), hepatitis B virus, HIV (papular pruritic emrption)
Bacterial: Group A beta-haemolytic streptococcus (scarlet fever, rheumatic fever), Nersseria meningitides (meningococcaemia), Salmonella typhi, Treponema pallidum (secondary syphllis), Mycobacterium tuberctlosr (papulonecrotic
tuberculosis), Lyme disease,Z isteria monoqttogenes
Rickettsial: Rickettsia conorii (South African tick-bite fever)
Other: Kawasaki disease,juvenile chronic arthritis
Diffuse erytbroderma (red skin)
Bacterial: Group A streptococcus (scarlet fever), Staphylococcas aureus (scalded skin syndrome), toxic shock syndrome
Fungal: Candida albicans (satelite nodules)
Other: Dnrg reactions
Vesicular, bullous, pustular
Viral: Herpes simplex virus type I and2, varicella zoster, coxsackievirus A
Bacteial: Staphylococcasaureus @ullous impetigo, scalded skin syndrome), group A sfeptococcus impetigo, Pseudomonas
aerugino sa (folliculitis)
Other: Toxic epidermal necrolysis, erythema multiforme (Stevens-Johnsonsyndrome), Behcet s1'ndrome
Petechial and/or purpuric
Viral: Enterovirus, atypical measles, congenital rubella or cytomegalovirus (CMV), viral haemorrhagic fevers e.g. CongoCrimean, Ebola, Lassa viruses
Bacterial: Meningococcaemia,also sepsisdue to other bacteria i.e. Streptococcus,Staphylococcusand other, infective
endocarditis
fuckettsial: Rickettsia conorii (South African tick-bite fever)
Other: thrombocltopenia, vasculitis, Henoch-Schdnleinpurpura
Urticarial
Viral: Ebstein-Barr virus, hepatitis B
Bacterial: Mycoplasma pneumonia, steptococcal infection
Erythemanodosum
Viral: Ebstein-Barr virus, hepatitis B
Bacteial: M. tuberculosis, group A streptococcus, Yersinia infections, cat-scratch disease
Fungal: Histoplasmosis,coccidiodomycosis, blastomycosis
Other: Systemic lupus erythematosus,inflammatory bowel disease,sarcoidosis
The other common appearanceis vesicular. The most well known vesicular
rash is due to varicella (chicken pox).
Others include hand-foot-and-mouth
diseasedue to coxsackieA virus and
also herpes simplex virus (HSV).
Distribution of rash
This may also give a clue. For example,
vesiclesin the mouth and on the hands
and feet are characteristicofhand-footand-mouthdisease.The maculopapular
rash of enterovirus and rickettsial diseases characteristically involve the
palms of the handsand sole of the feet.
The purpuric lesionsof Henoch Schcinlein purpura are classicallymost prominent on the distal aspectsof the lower
limbs and lesions above the buttocks
are rare.
Varicella zosteroccursmore commonly in immunocompromisedthan
immunocompetent children and an underlying causefor immunosuppression
shouldbe excluded.It may also disseminate in immunocompromised children.
Herpetic lesionscausedby HSV also
causedisseminatedvesicles in immunocompromisedchildren.
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Progression
Of note is that the characteristicblack
appearanceis not immediately apparent
Many rasheshave characteristicfeatures
and
appearswith time. The lesionsvary
and diagnosescan be made with relative
in
size
and may be only millimetres in
certainty. Specific aspectsofrashes in
Failure to recognizethese
diameter.
children are shown in table I. An imfeatures
might
causea delay in treafrnent
portant and yet often neglected feature
might
have
fatal consequences
and
of a rash is its natural history. For exRashesfrom most viral illnesses
ample,a petechialrash or even isolated
progressover hoursto days.For exampetechial spots (macules that do not
ple, in measles,the rash startsbehind
blanche on pressure),papules or more
the ears after a prodrome of three to
rarely a maculopapularrash,progressing
four days (fever, coryza,conjunctivitis,
rapidly with new spots appearingover
and Koplik spots), gradually
cough
minutes and hours in a toxic child is
progresses
to the trunk and limbs over
almost certainly meningococcaemia
the
next
two
to three days, resolving
(figure 2).
over the next two to t}ree daysin reverse
sequencewith fading of the rash and
desquamation.
In infective endocarditis,development of new lesionsmay be episodic
over days (figure 3). Lesionsarewell
circumscribed (figure 3). Failure tc
auscultate the heart in a febrile chilc
may contribute to progressivevalvt
damage,againwith fatal consequences
Cutaneoustuberculosis,(papulonecrotir
tuberculosis),persist for weeksandma'
be prominent on the ears(figure 4). l
Figure 2 : Meningococcaemia showing
juvenile chronic arthritis with systemi
small well circumscribed purpuric lesions
onset, a pathognomonic featureis th
in 9 month-old infant on legs
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presenceofa generalizedpapulareruption only during fever.
Physical signs that assistin establishing the cause of a rash
Figure 3: Endccarditis showing focal skin
infarcts
A number of exanthematousdiseases
in children have distinctive features
where failure to make a diagnosis has
serious consequencesfor the child or
the community. Examples include Kawasaki disease(KD) and measles.With
KD, there is a 20 percent chance of
developing coronary aneurysms if correct treatment is not given early in the
diseaseand for measles,failure to identifu and prevent spread ofthe disease
will promote the spread in vulnerable
infants especially if immunocompromised or under 9 months of age. South
African tick-bite fever presentswith a
maculopapular rash often involving the
handsand feet.
spection ofother sites such as the external auditory canal,axillae and perineum.
The escharmay be easily overlooked,
especially if the skin is pigmented and
a thorough search is not made (figure
5). Occasionally,there is no eschar,but
this should not prevent the institution
of appropriate therapy.
Mouth and lips
A strawberry tongue is seenwith infection by group A beta-haemolytic Streptococcus, KD and toxic shock syndrome. Initially, if the tongue is coated,
with inflamed papillae peeping through,
the condition is termed a "white strawberry tongue" and once the coating has
disappeared,a "red strawberry tongue"
(figure 6).
Eschar
Figure 4: Papulonecrotictuberculosis
IMell circumscribed versus coalescent
lesions
One useful distinguishing feature between viral and bacterial (or rickettsial)
skin lesionsis that that viral rashesoften
coalescewhereasin bacterial infections,
the papulesare often very distinct. Exceptionsare toxin-mediated skin lesions
from toxic shock syndrome(TSS), staphylococcal scaldedskin syndrome and
scarlet fever, where the skin manifestations are extensiveand coalescent(and
intenselyerythematousin nonpigmented
skin). Occasionally,the rash may also
be coalescentin severerickettsial or
bacterialinfections. On the other hand,
the maculopapularrash associatedwith
rubellais not coalescent.
HIT/
With the adventofHIV disease,children
are presentingwith new expressionsof
dermatologicaldisease.Most conditions
are seenin children without HIV infection as well, but are more intense and
more extensive in HlV-infected children.Conditions include papularpruritic
eruptionand severescabies,often alone
or in combinationand easyto confuse.
Herpetic infections are also more common and more extensive.
Tick-bite fever presentswith headache,
fever and a papular rash a week after
the tick bite. Often, the child may have
been camping or hiking on the previous
weekend. Once the eschar has been
located, the diagnosis is obvious and
specific therapy can be instituted. The
eschar is commonly found above the
hairline.
Figure 6: Red sfrawbeny tongue in a
patient with scarletfever- white coating
seencentrally.Notc alsothe erythematous
rash,lessprominentbecauseof the
pigmented skin
Figure 5: Escharassociatedwith South
African tick bite feverA). abovethe
hairline and B). on the shoulder(alsonote
characteristicwell-circumscribedpapules
- thick arrow)
Iftick bite fever is suspected,and the
escharis not found above the hairline,
the clinician should make a careful in-
Other conditions associatedwith mucositis include KD, often reflected by
extremely inflamed lips that then become chapped and StevensJohnson
syndrome (figure 7).
Figure 7: Cheilosisassociatedwith
Kawasakisyndrome
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Koplik's spots in the early phase of
measleshas already been mentioned.
The dermatosis associatedwith protein
energy malnutrition (hyper and hypop'ocrazy
igmented areas and
paving"dermatosis)may be associated
with angular stomatitis.
pular rash (figure 9). While trivial in
most patients,it can be associatedwith
erythroblastosis foetalis if a pregnant
susceptiblewoman is exposedand is
also associatedwith aplasticcrisesin
children with haemolytic anaemia.
vasculitic diseasecausedby Pseudomonasaeruginosa,occursin the inguinal area. This condition requires
early recognitionand specificantipseudomonal treatment.Failure to examine
the nappy areatn a critically ill patient
will result in serious consequences
(figure 10).
Desquamationof skin
Desquamation,especiallyof the hands
is characteristicof a number of conditions, especiallywhere bacterialtoxins
have been implicated. Conditioni include TSS, KS and scarlet fever. The
most prominent site for desquamation
is on the hands. In KD, especially,the
desquamation can be extremely subtle,
occurring at thejunction ofthe nail bed
and hngertip (figure 8). Occasionally,
it may occur in other areas,for example,
in the groin (KD) or even on atypical
areassuch as the knees.
Figure 8: B
due
Figure 10: Erythemagangrenosum
to Pseudomonasaeruginosiain an infant
with acute lymphoblastic leukaemia
Lymphadenopathy,hepatomegalyand
splenomegaly
Figure 8: A. Desquamationof skin on
fingertips (Kawasakisyndrome).Notethe
swollendigits also. B. Toxicshock
on
with desquamation
syndmmeassociated
hands (typical site) and C. knees
Figure 9: Parvovirus infection is
associated with A (above) a "slapped
cheek" appearance and B (below)
reticular ttlaceJikett rash
I
li
The nappy area
Figure 8: B
" Slappedcheek" appearance
ParvovirusBl9 causesa red appearance
of the cheeks and is often associated
with a reticular "lattice-like" maculopaSA Fam Pract 2oo3;45(ro)
Nappy rashesoccur commonly and are
usually well managed. The satellite
lesionsassociatedwith candidainfection
are well known and easily managed.
Rarely, in immune compromisedinfants
ecthyma gangrenosum,an aggressive
Infectious mononucleosis@bsteinBarr
virus) presentswith a combination of
lymphadenopathy,hepatosplenomegaly,
palatal petechiaeand a maculopapular
rash. The rash is usually elicited or
accentuatedby ampicillin.
HIV causesmultisystemdisease
and is commonly associatedwith lymphadenopathyand hepatosplenomegaly.
Becauseof progressiveimmune dysregulation, intercurrent pathological infecmay
tious and non-infectiousprocesses
be more severe.For example,varicella
is more severe.Scabiesis also more
severeand is also commonly confused
with papular pruritic eruption of HIV.
Healing is associatedwith pigmentation,
leading to extensive hyperpigmented
maculeswith widespreaddistribution.
Papular urticaria may be related to or
confusedwith insectbites (figure 11).
Psoriasismay be seenin childrenwith
HIV, with skin lesionsbeing confused
asnon-resolvingfirngal infections.Clues
to psoriasis may be the presenceof
arthropathy and pitting of the nails.
Vesicular rashes
Chickenpox usually does not present
diagnosticproblems.The lesionsbegin
as maculesand rapidly progressto vesJJ
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iclesand crusts.All stagesof the lesions
are presentat any one time. Usually less
than 500 lesions occur over the course
of 2 to 3 daysbut occasionallythe eruption may be worse (figure 12).
Figure 14: Nevirapine-associated
rash
Figure 11:Papularlesionspossibly
associated
with fleabitesin a HlV-infected
infant on cotrimoxazoleprophylaxis for
6 months.Note the linearity of the lesions
and alsoolder lesion(thick arrow)
Figure 12:A. Severechickenpox in a 10
year old girl.
Stevens-Johnsonsyndrome and toxic
epidermalnecrolysisis associatedwith
a rash, usually described as "target
lesions".Although lesionsmay initially
appearpapularthey may rapidly become
bullous and after rupture or resorption
offluid" may appearvasculitic(fuure 13).
Figure 12: B. Severechickenpox in a
HlV-infected infant. Note the different
stagesof lesions
effect. For example, it is often seen
within the first 6 weeks of using nevirapine (NVP), a non-nucleosidereverse
tanscriptase inhibitor for HIV infection.
Although this drug is used in combination with two nucleosidereversetranscriptaseinhibitors, the rash is so characteristic for NVP, that it, alone, can be
substitutedifthe rash is severe,progressive, associatedwith mucosal changes
or raised liver enzymes. A NVPassociatedrash is shown in figure 14.
nile chronic arthritis has already been
mentioned and should be carefully
sought when the patient is febrile. Becausejuvenile chronic arthritis is often
associatedwith prolongedfever, expensive investigationsareundertakento find
a source.By observing the rash, much
expenseand anxiety can be avoided.o
Non-infectious causesof skin rashes
Drug reactions
Any drug, somemore often than others,
such as antibiotics (penicillin derivatives, sulfonamides),antituberculotics
(isoniazid, fluoroquinolones)and anticonvulsants (barbiturates, carbamazepine),may be associatedwith a
rash. A history of drug exposureis extremely important. The type of rash
varies but is most commonly maculopapular and is often itchy. With some
medications.skin rash is a common side
Figure 13: Toxicepidermalnecrolysis.
Fluid-filled blistersand lesionsafter
reabsorptionoffluid.
Collagen vascular disease
Collagen vascular diseasesoften have
skin manifestations.Probably the best
known of these is the butterfly rash or
small vesselvasculitis associatedwith
systemic lupus erythromatosis (SLE)
and discoid lupus. The maculopapular
eruption seenin systemic onset ofjuve-
Recommended Reading
l. Mandell, Douglas and Bennett's
Principlesand Practiceof Infectious
Diseases5th ed 2000, Churchill
Livingstone, Philadelphi4 PE, USA
2. American Academy of Pediatrics.
Pickering LK ed. 2000 Red Book:
Report of the Comnlittee of
Infictious Diseases,25thed. Elk
Grove Village, IL,: American
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