Case 11: 5yo - Kawasaki, other weird rashes Flashcards
diffdx of irritability in a child
==> pain / discomfort from any source
- meningeal irritation
- HA from intracranial irritation (or increased intracranial pressure)
- simple exhaustion
diffdx for rash + fever - what broad categories should we be thinking of?
INFECTIOUS
- Enterovirus
- measles
- meningococcemia
- erythema infectiosum
- roseola
- scarlet fever
- varicella
- osteomyelitis / septic joint
- Rocky Mountain spotted fever (RMSF)
IMMUNE
- systemic-onset juvenile idiopathic arthritis
- Kawasaki disease
- Stevens-Johnson syndrome
Enterovirus rash (hand-foot-mouth)
Causative agent: location:
description:
Timing:
Fever:
What is this rash?
Causative agent: Coxsackie
location: palms & soles +/- petechial; ulcers in the mouth
description: erythematous, maculopapular, vesicular
Timing: late summer, early fall
Fever: 3-5days, HIGH
Rash is NOT painful and should not cause refusal to walk.
Erythema infectiosum (fifth disease) Causative agent: location: description: Timing: Fever:
What is this rash?
Causative agent: Parvovirus B19
location: face
description: face = “slapped cheek”; body = erythematous macular; with central clearing - ‘lacy’ appearance. Lasts longest in extremitites
Timing:
Fever: low-grade fever
OTHER: pain & swelling in joints = polyarthropathy syndrome
1) fever
2) 7-10d - rash “slapped cheek”
3) rash spreads to trunk
Measles Causative agent: location: description: Timing: Fever:
What is this rash?
Causative agent: location: neck, behind the ears, along hairline
description: maculopapular, with buccal mucosal red lesions + bluish white spots in center (Koplik spots).
Timing:
Fever:
1) Eyes w/ conjunctivitis
2) Neck, behind the ears, along hairline
3) 2-3d - spread downward, reaching feet
What else besides rash & fever can someone with parvovirus B12 present with?
polyarthropathy –> most likely in adults
pt with immuno deficiency + infection with ParvoB12 - what is the risk?
aplastic anemia
Pt presents with very classical measles story and spread of rash from top to bottom. However, he does not have the Koplik spots. What’s going on?
(do you know what Koplik spots look like)?
Koplik spots = buccal mucosal red lesions + bluish white spots in center
This the initial rash. they have frequently disappeared by the time the pt presents to medical attention
Meningoccocemia
Causative agent: location: description: Timing: Fever: Other
What is this rash?
Causative agent: Neisseria meningitidis location: generalized description: (1) urticarial, maculopapular, petechial (hemorrhagic spots) --> (2) fulminant, purpuric, large hemorrhages on skin Timing: Would arise quickly (<3d) Fever: Abrupt \+ chills, malaise, prostration
Prevention = immunization
Roseola (exanthem subitum)
Causative agent: location:
description:
Timing:
Fever:
What is this rash?
children < 2yo
Causative agent: 6th disease; HHV6 location: (1) trunk, (2) arms & neck
description: maculopapular rash
Timing:
Fever: HIGH
1) High fever = 38.5-40.5, +/- rhinorrhea
2) 3-4d - rash (no fever) + resolution of fever
3) bulging fontanelle
+/- febrile seizure
Scarlet fever
Causative agent: location:
description:
Timing:
Fever:
What is this rash?
Causative agent: Group A strep location: (1) groin, axillae, neck –> (2) generalized
description: very fine papules (“sandpaper”)
Timing:
Fever: HIGH, <5d
self-limited = lasts 10d (5d fever, 5d rash) Tx = Bacitracin / penicillin Complications = non-suppurative complications (rheumatic fever)
Varicella (chicken pox)
Causative agent: location:
description:
Timing:
Fever:
What is this rash?
Causative agent: location: (1) trunk –> (2) extremities, head
description: (1) erythematous macule –> (2) papule –> (3) vesicle –> (4) pustule –> (5) crusted. Various stages of development in the same area
Timing:
Fever: Mild
self-limited; lasts 1w
Prevention: immunize
diffdx diffuse lymphadenopathy +/- splenomegaly
INFECTIOUS
- measles –> marked generalized lymphadenopathy & splenomegaly (lasts for several weeks)
- infectious mononucleosis –> by EBV or CMV
- HIV
- histoplasmosis
- toxoplasmosis
- mycobacteria –> localized lymphadenopathy
NON-INFECTIOUS
- lymphoma
- leukemia
- histiocytosis
- metastatic neuroblastoma
- rhabdomyosarcoma
what’s worse - diffuse lymphadenopathy or isolated enlarged node
diffuse lymphadenopathy
diffdx unilateral cervical lymphadenopathy
INFECTIOUS
- oral inflammatory / infectious reactive lymph node
- bacterial cervical adenitis
- cat scratch disease
- mycobacterial infection
NONINFECTIOUS
- Kawasaki disease
bacterial cervical adenitis
WHAT IS THIS U/L cervical LYMPHADENOPATHY
Age: 1-5yo; h/o recent URI
ORGANISM: Staph aureus, Strep pyogenes
sxs - HIGH fever, toxic
cervical lymphadenopathy + overlying cellulitis & development of fluctuance
diffdx for “strawberry tongue”
“strawberry tongue” = erythematous tongue with prominent papillae –> due to desquamation of minor papillae & subsequent prominence of major papillae (strawberry red with white “flecks”)
- strep pharyngitis +/- infectious mononucleosis
- Kawasaki disease
- toxic shock syndrome
diagnostic criteria for kawasaki disease (sxs)
1) HIGH FEVER for >/- 5d
2) Changes in oral mucosa (“strawberry tongue”) - NON-PURULENT
3) extremity changes (acute - swelling; chronic - redness/dequamation). PAINFUL & REFUSAL TO WALK
4) U/L cervical lymphadenopathy
5) rash in groin & perineum; palms & soles (non-pruritic; palpable).
6) conjunctival injection (“limbic sparing = radiating red lines out from iris except for where iris meets with sclera) - NON-PURULENT
non-indicative of infectious / bacterial etiology
diagnostic criteria for kawasaki disease (acute labs)
–> high ESR, CRP
1) high ALT
2) high WBC (neutrophils); nml MCV
3) high Plts [esp. in chronic, @ 2-3w]
4) low Hgb (norm,norm)
5) sterile pyuria ==> with irritation at the urethra (not bladder)
6) hypoalbuminemia
Complications of Kawasaki
1) CNS = irritability, lethargy, aspetic meningitis (90%)
2) coronary aneurysm (25% in untreated)
3) liver dysfunction (40%)
4) arthritis (30%)
5) hydrops of the gallbladder (10%)
6) Effusions (pleural, pericardial).
In a child with Kawasaki’s, what’s the timeline of presentation for a coronary aneurysm
end of 1st week - 4w after onset of disease
Management of Kawasaki
1) IVIg
2) ASA (high dose until fever breaks, then low dose 6-8w)= to minimize risk of coronary aneurysm
3) Echo during acute phase to look for aneurysm (diagnostic and monitoring)
4) Watch for bleeding, stomachache, blood in stool.
5) flu vaccine
6) Pain - prescribe anti-inflammatory meds; physical therapy
7) monitor for fever
8) f/up at 1-2w for repeat ECHO (since coronary artery aneurysms, if they develop, do so within 4w).
9) f/up in another 2w for another ECHO
–> then no more if all is normal
define: macule
small
flat, discolored spot
define: papule
small
well-defined solid bump
define: vesicle
small
well-defined solid bump + fluid
define: pustule
small
well-defined solid bump + pus
define: plaque
small
raised, differentiated patch / area on body surface
define: desquamation
shedding of outer layer of skin surface
systemic-onset JIA description: Fever: Rash: Other
What is this?
description: rheumatic disease (oligo- or poly-arthritis, systemic onset disease = Still’s disease)
Fever: prolonged fever (suggestive of Still’s disease)
Rash:
Other: Arthritis (usually months to years after onset); visceral (hepatosplenomegaly, lymphadenitis, serositis)
Kawasaki disease Description: Fever: Rash: Other
What is this? Description: Vasculitis Fever: 4-5d Rash: Other: non-purulent conjunctival injection; selling & erythema of extremities
Osteomyelitis / septic joint Description: Fever: Rash: Other
What is this? Description: Bacterial infection 80% in LE Fever: low grade Rash: around knee + warmth Other: painful walking
Rocky Mountain spotted fever Description: Fever: Rash: Other
What is this?
Description: tick-borne bacterial infection (Rickettsia Rickett
Fever:
Rash (95%): (1) ankles, wrists –> centrally –> palms & soles. Maculopapular –> petechial
Other: Myalgia
Diffdx of rash on palms and soles
- Kawasaki
- enterovirus
- syphilis
- Rocky Mountain spotted fever
Stevens Johnson syndrome Description: Fever: Rash: Other
What is this?
Description: mucocutaneous d/o –> d/t hypersensitivity rxn to meds, infections, other illnesses
Fever: <7d
Rash: severe, pruritic (= ERYTHEMA MULTIFORME)
Other: mucosal changes; conjunctivitis
A 5yo pt presents with rash that is on his palms and soles. He cries when you touch it, and his parents have to carry him to your office. what is it?
(and what is it not?)
Kawasaki == kids find it painful, to the point of no wanting to walk
NOT enterovirus
Diffdx for erythema, pain and swelling of an extremity
- Kawasaki
- (if + joint) arthritis
- Cellulitis (unless multiple extremities are involved)
- (if + multiple extremities) consider erythema multiforme
workup of Kawasaki dz
1) CBC = low Hgb, high WBC, high Plts
2) Blood culture (to r/out bacterial illess)
3) LFTs = elevated AST, ALT, low Alb
4) elevated ESR, CRP (nonspecific).
5) UA (clean catch urine)- sterile pyuria (urethritis) = 5-10WBCs
6) Echo for baseline / search for coronary aneurysm.
In which of the diffdx for fever + rash would you see elevated liver enzymes?
Kawasaki
Stevens Johnson syndrome
when is getting ESR and CRP in Kawasaki helpful?
Not in the acute phase b/c it’s nonspecific
if ESR negative ==> can r/out Kawasaki
if ESR elevated after fever has subsided -==> can distinguish Kawasaki disease from other infectious rash/fever illnesses
A 7yo pt comes in with what you think is Kawasaki disease. You had ordered a UA, and your nurse asks you if you want a clean-catch urine or via straight catheter. which do you choose and why?
clean catch b/c it is more likely to show white cells (since Kawasaki shows a sterile pyuria 2/2 sterile urethritis)
A catheterized urine will NOT show it, because the bladder is fine in kawasaki
why is it important to diagnose pharyngitis 2/2 group A beta-hemolytic strep (as opposed to any other etiology)?
b/c complication of rheumatic fever
diagnostic tests for group A beta-hemolytic strep
1) “rapid strep” teest –> strep antigen from swab of tonsils and posterior pharynx
2) if NEGATIVE ==> throat culture
If you suspect group A beta-hemolytic strep (but have no documentation yet), should you treat it?
What with?
1) should document GAS, then treat
2) IF there is strong pretest probability (sick contacts at home, high score with only sore throat and no other sxs, etc.) –> can treat based on clinical diagnosis
3) If neg rapid strep - then culture - then treat
treat within 9d from start of acute illness
Treatment options
1) Oral amoxicillin (10d)
2) IM penicillin (1 dose)
3) oral penicillin (b/c the taste sucks)
treatment of Kawasaki disease
1) IVIg - single dose of 2g/kg over 10-12h –> decreased incidence of coronary artery aneurysm
2) Aspirin
(a) high dose (80-100mg/kg) ==> for anti-inflammation to decrease fever
(b) low dose aspirin (3-5mg/kg) ==> for antiplatelets for a total of 6-8w if no coronary changes seen in f/up echos.
if + coronary artery abn –> lifetime low dose aspirin
you know that aspirin and Kawasaki is important. So why do you have to worry about flu if you are sure that this is Kawasaki?
for patients who are diagnosed with Kawasaki (especiallly with long-term treatment with aspirin - in 6-8w or lifelong) –> physicians have to be vigilant about recommending influenza vaccination
Can you give ibuprofen and aspirin at the same time in Kawasaki for antipyretic effects?
NO
1) other antipyretics (v. aspirin) have not been effective for fever control
2) ibuprofen antagonizes the irreversible platelet inhibition induced by aspirin ==> AVOID in children esp. with coronary aneurysms taking aspirin for antiplatelet effects
when should y ou be suspicious for kawasaki, but don’t have the slam dunk diagnosis?
“incomplete” kawasaki = 3/5
1) Infants ≤ 6 months old on day ≥ 7 of fever without other explanation should undergo laboratory testing and, if evidence of systemic inflammation is found, an echocardiogram
2)
for which of the following sxs should a syndrome other than kawasaki be considered? (may choose more than 1; explain) a. exudative conjunctivitis b. exudative pharyngitis c. discrete intraoral lesions d. bullous or vesicular rash E. generalized adenopathy
All of the above
kawasaki should not be purulent
U/L
if you suspect kawasaki, which should you do first?
a. treatment with IVIg and ASA
b. echocardiogram to look for coronary artery aneurysm
A
realistically, it takes a while to get the echo, and in the meantime, you can just start the meds
what are the conditions for a echo positive for kawasaki?
coronary artery aneurysm
1) Enlargement (increase in angle) of LAD, RA for z-sore >/= 2.5
2) coronary aneurysm found
3) >/= 3 other suggestive features exist = perivascular rightness, lack of tapering, decreased LV fx, Mitral regurgitation, pericardial effusion, z-score of LAD, RCA of 2-2.5
Of the following possible complications, which ONE requires evaluation in every patient with Kawasaki disease?
A Aseptic meningitis B Coronary artery aneurysms C Liver dysfunction D Arthritis E Hydrops of the gallbladder
each of these happen, but only B requires evaluation in every pt
A 5-year-old female, previously healthy, presents with an erythematous, vesicular rash on the palms and soles and a high fever for several days. Upon examination, she is also found to have ulcers in her mouth. A few days later, the fever and rash resolve. What is the most likely pathogen?
A Herpes simplex virus 1 (HSV-1) B HIV C Enterovirus D Human herpesvirus 6 (HHV-6) E Group A strep
C. cocksackie A, an enterovirus. Following an incubation period of three to five days, patients have fever, tender vesicles on their hands and feet, and oral ulcers. Sometimes the rash also occurs on the buttocks and the genitals. The infection resolves spontaneously within three days, and is spread from person to person via saliva, fluid from the vesicles, stool, or nasal discharge.
HSV1 - gingivostomatitis + fever and malaise == but no lesions on hands and feet
HIV = no lesions on hands and feet
HHV6 = roseola: fever THEN macular / maculopapular rash - but it begins on the trunk –> then to extremities, NO ORAL LESIONS
Group A strep - scarlet fever - more “sandpaper,” and it’s all over
A 2-year-old girl presents to the urgent care clinic with a 7-day history of high fever to 38.5 C, a maculopapular rash that began on the palms and soles of her feet, red eyes without discharge, and unilateral cervical adenopathy. What other symptom/sign might you discover on further history and exam?
A Tonsillar exudates B Headache C Erythematous and edematous feet D White spots on buccal mucosa E Dysuria
C - Kawasaki –> + strawberry tongue
If tonsillar exudates –> strep paryngitis / tonsilitis [the mentioned is too widespread]
if HA –> rocky mountain spotted fever [only if with fever & rash in palms & soles]
If white spots on buccal mucosa (Koplik spots ) –> measles [but would also have cough, coryza; rash usually head down]
if dysuria –> UTI [the mentioned has too many sxs of something else.]
A 3-year-old male presents with fever to 103 F for the past week, injected eyes, and a refusal to walk for the past two days. On physical exam, you note conjunctival injection without pus or exudates bilaterally, prominent papillae of his tongue with redness as well as redness of his hands, and feet. He also has a new non-diffuse maculopapular rash on his torso that gets worse with fever. On examination of the swollen extremities, you are unable to elicit any tenderness or effusions in any joints. Which of the following is the most likely diagnosis?
A Rocky Mountain Spotted Fever (RMSF) B Bone or joint infection C Kawasaki disease (KD) D Scarlet fever E Systemic onset juvenile idiopathic arthritis
C
likely “incomplete” Kawasaki b/c doesn’t have U/L cervical lymphadenopathy
Rocky mountain spotted fever = headache, fever, myalgia, and a centrally progressing petechial rash originating on the wrists and ankles.
Bone / joint infection (makes sense with refusal to walk) == but should have lower fever, with warmth / tenderness / effusion
Scarlet fever = sore throat, fever, “strawberry tongue” and a blanching, erythematous rash with desquamation of the affected areas about six to seven days later as the rash begins to disappear.
SJIA = should have hx of spiking fevers and “salmon” rash occurring when child is febrile; disappearing as fever fades. Also should have tenderness & effusion of joint.
A 5-year-old male comes to the clinic with a chief complaint of four days of progressively worsening fever and that has been minimally responsive to acetaminophen. The patient complains of sore throat and decreased appetite. His sister had a positive rapid strep test and is now being treated with amoxicillin. Your concern is for Group A strep. What is the next best step in management?
A Start antibiotic treatment
B Send blood cultures
C Advise parents to give patient acetaminophen with return precautions
D Rapid strep test with back-up culture if negative
E Chest x-ray
1 - do the test –> to allow for correct diagnosis prior to empiric antibiotic treatment
D
A 3-year old girl comes to the clinic with a chief complaint of fever (104F) for over a week. Her mom reports that she has been fussy and inconsolable since she became febrile. She has a red tongue, with large papillae, conjunctivitis, a palmar rash, unilateral cervical adenopathy, as well as swollen feet. Given the most likely diagnosis, what is the most important follow-up for this patient over the next few weeks?
A Ophthalmology follow-up to determine extent of eye damage and determine need for corticosteroids
B Physical therapy follow-up to help prevent long-term joint deformities and ensure long-term functionality
C Cardiology follow-up to rule out presence of rheumatic fever
D Echocardiogram to look for coronary artery aneurysm
E Neurology follow-up to evaluate partial paralysis of lower extremities
D. Kawasaki disease are at high risk for coronary artery aneurysm formation and should receive an echocardiogram within four weeks of the onset of their illness. Use of IVIG for the treatment of Kawasaki disease has decreased the risk of coronary artery aneurysms significantly. Kawasaki disease is diagnosed when there is a fever plus four of the following: changes in oral mucosa (e.g., strawberry tongue), extremity swelling or redness, unilateral cervical adenopathy, conjunctivitis, and rash. Infectious and rheumatologic causes must be excluded in order to make the diagnosis of Kawasaki disease.
NOT concerned for rheumatic fever b/c would be Scarlet fever = erythematous, blanching, sandpaper-like rash” with very fine papules secondary to infection with Group A streptococcus. It may start in the groin, axilla, or neck, before spreading rapidly over the trunk and extremities. Fever can be high, but generally resolves within five days.
How to describe a rash
- anatomic location, distribution (generalized / localized?)
- color (hyper/hypopigmentation, erythema, purpuric / ecchymotic)
- morphology (pattern and shapes - annular, discrete, clustered, confluent, dermatomal, eczematoid, guttate, target, linear, multiform, reticular, serpigious, universalis)
- size
Types of primary lesions
Macule Papule Nodule Tumor Plaque Vesicle Bullae Pustule Wheal Burrow Telagiectasia
types of secondary lesions
Scale Crust Atrophy Lichenification Erosion Excoriation Fissure Ulceration Scar Eschar Keloids Petechiae, purpura, ecchymoses
6 month old presents with what you think is roseola. however, he also has a bulging fontanelle. should you be worried?
bulging fontanelle is an unusual finding of roseola
==> should r/out meningitis
how are HHV6 and febrile seizures related?
HHV6 fever is HIGH (38.5-40.5)
primary HHV6 infection associated with ~20-30% of first febrile seizures in children
