exanthemas
Post on 06-Aug-2015
50 Views
Preview:
TRANSCRIPT
Exanthemas in children
Definition
• Exanthemas is a group of diseases with clinical presentations of rash
• “Exanthema” – rash on the skin
• “Enanthema” – rash on mucous membranes
Groups of diseases with exanthemas
• Infectious
• Allergic
• Dermatological
• Autoimmune
• Toxic
• Neurological
• Idiopathic
Infectious causes
1. Namely exanthematous diseases (exanthema is a key presentation of the disease) = childhood exanthemas
2. Diseases, accompanied by exanthema syndrome
Classification scheme of childhood exanthems
1. Measles
2. Scarlet fever
3. Rubella
4. Filatov-Dukes disease (an atypical scarlet fever)
5. Erythema infectiosum = fifth disease, parvoviral B19 infection
6. Roseola infantum = sixth disease, HHV-6
Other infectious diseases with exanthema syndrome
• Chickenpox • Enteroviral infection• Pseudotuberculosis• Meningococcemia• Infectious mononucleosis (beta-lactame
penicillines)• Syphilis • Rocky Mountain spotted fever• Leptospirosis, etc.
Differential features of infectious exanthemas
Features to help differentiate infectious origin of rash from non-infectious
Presence of feverAppearance of symptomsOther clinical symptomsCharacter of rashEpidemiological contactsLaboratory methods, etc.
Types of rash of infectious origin
• Roseolar rash
• Papullar rash
• Small roseolar (pointed) rash
• Macular rash
• Erythema
• Vesicular rash
ROSEOLAR RASH Roseola is a small macula (d=2…5 mm) of pink, red color, often of round shape. it presents widening of the papillary layer of the skin. Disappears at pressing or skin stretching
• INFECTIOUS DISEASES• Roseola infantum• Parvovirus infection • Pseudotuberculosis • Leptospirosis • Typhoid fever • Paratyphoid fever А & Б• Secondary Syphilis
• NON-INFECTIOUS DISEASES
• Insect bites
SMALL ROSEOLAR (POINTED) RASH
Small in diameter, 1 mm, roseolar red elements, which also disappear on pressure. Slightly elevated over the skin, often
appear on hyperemic background.
• INFECTIOUS DISEASES• Scarlet fever • Staphylococcal infection • Kawasaki disease• Pseudotuberculosis
NON-INFECTIOUS DISEASES
• Medicament rash • Toxicodermia
MACULAR RASHMACULE is an element similar to roseola, but larger (5…20 mm), not elevated over the skin. There is «small macular»
(5 – 10 mm) and и «large macular» (11-20 mm), which is used for differential diagnosis.
INFECTIOUS DISEASES• Measles • Rubella • Infectious mononucleosis• Enteroviral infection • Parvoviral infection• Pseudotuberculosis • Leptospirosis • Endemic(tick-born) recurrent fever• Epidemic(louse-born)relapsing fever
NON-INFECTIOUS DISEASES
• Secondary Syphilis• Medicament rash
PAPULAR RASHPAPULE is a non-cavity superficial rash, elevated over the skin.
Caused by epidermis proliferation and papillar infiltration with vessels widening and localized edema.
• INFECTIOUS DISEASES
• Measles • Infectious mononucleosis• Enteroviral infection • Parvoviral infection• Pseudotuberculosis • Leptospirosis
• NON-INFECTIOUS DISEASES
• Secondary Syphilis• Allergic dermatitis
ERYTHEMA RASHERYTHEMA is a non-cavity superficial hyperemic rash , caused by papillar edema and increased blood supply.
INFECTIOUS DISEASES
• Parvoviral infection
• Scalded skin syndrome
• Kawasaki• Staph and Strep
toxic shock • Scarlet fever • Lyme disease • Cellulitis • Erysipelas
NON-INFECTIOUS DISEASES
• Allergic dermatitis• SLE • Rheumatic fever
Sclerodermia • Toxic dermatitis• Burnt skin • Toxicodermia of
newborns • Layella syndrome
VESICULAR RASH Vesicles are small rash with cavities filled with
serous, or serous-hemorrhagic fluid
INFECTIOUS DISEASES• Varicella• Herpetic infection, caused by HSV 1 & 2 types• Herpes zoster
HEMORRHAGIC RASH Rash does not disappear at stretching the skin,
can be small (petechiae) or large, can be accompanied by necrosis
INFECTIOUS DISEASES• Meningococcemia• Seldom – pneumococcal infection
Criteria of rash description for exanthemas diseases
1. Character of the elements2. Prevalent localization3. Background skin color4. Time of appearance5. Connection to fever6. Itching 7. Fusion of rash elements 8. Step-wise appearance9. Presence of enanthema10.Disappearance of the rash
MEASLES
MEASLES
Viral infection with airborne way of transmission, cyclical course and presence of syndromes of intoxication, catarrhal inflammation and exanthema.
History
• Before antibiotics - «children’s plague»: only during 1910th in Europe about 1 million deaths due to measles, mostly due to bacterial complications.
• After antibiotics (after 1940th) mortality decreased considerably, but morbidity was still high.
• After vaccination (since 1960th) morbidity also dropped considerably.
Etiology
• Virus was isolated in 1954. • RNA-containing • Paramyxoviridae, genus Morbillivirus• Virus antigenically identical all over the world →
repeated cases only in 0,5-0,3%• Virus is not stable • Highly transmittable • Causes appearance of multinuclear giant cells in
body, e.g., measles pneumonia
Epidemiology• Way of transmission – airborne • Incubational period – 9-17 days (21 if human Ig injected) • The source of infection patient from 2 last days of
incubational period and till 4 days after appearance of rash (10 days in case of complications)
• Maximal contagiosity – during catarrhal period • Index of contagiosity is 100% • Currently, after vaccination, more cases among adults
are seen • Case fatality rate is mostly due to early age children and
cases of encephalitis • Main cause of acquired blindness in children in
developing countries
Pathogenesis
• Entry of infection – respiratory mucous. • First virusemia – on 3rd day of incubational period
(→ anti-measles Ig is effective during first 3 days) • Virus damages respiratory mucosa • Respiratory tract damage and immunosupression
→ bacterial infections• Pneumonia can be early (viral) or late (bacterial) • Immunosupression is seen some time after
recovery
Clinics – periods
1. Incubational period - 9-17 days (21 day after injection of Ig)
2. Catarrhal period – 3-4 days. Syndromes of intoxication and catarrhal inflammation (of respiratory tract, conjunctiva, intestinal). Dry cough, rhinitis, conjunctivitis, slight face edema.
3. Rash period - since 4th-5th day. Intoxication and catarrhal syndrome still increase. Croup or dyspepsia in young children can appear
4. Pigmentation period
Clinics
Pathognomic sigh – Filatov-Koplik spots: whitish small points on the inner cheek mucosa at the level of premolares, not removable (focal necrosis of epithelium). Appears on the 1st day of the disease, disappears by the 3rd day. Can be on epithelium of conjunctiva and vagina
Filatov-Koplik spots
Clinics – rash characteristics
1. Character of the elements – macular-papulous 2. Prevalent localization – all over the body 3. Background skin color – normal 4. Time of appearance – 3rd – 4th day of the disease 5. Connection to fever – intoxication increases 6. Itching – very rare7. Fusion of rash elements – yes 8. Step-wise appearance – 3 days (face → trunk →
extremities) 9. Presence of enanthema – Koplik spots (first 3 days) and
palate enanthema (concurrent with rash) 10.Disappearance of the rash – pigmentation
MEASLES
Classification
1. Typical (mild, moderate, severe) and atypical (abortive, mitigated, low-grate, asymptomatic) – 5-7%.
• Mild: fever under 38,5С, mild intoxication and rash
• Moderate: fever 39С, vomiting, prominent rash• Severe: seizures, loss of consciousness, fever
40 and higher
2. With / without complications
Abortive and mitigated forms
• Abortive and mitigated forms: catarrhal period is 1-2 days or absent, almost no intoxication. Rash is 1-2 days, pale, scanty, without step-wise appearance, mild pigmentation. Koplik spots can be absent. Develops after injection of Ig, blood preparations or incomplete vaccination.
Complications
1. Etiology:
• viral (measles virus)
• bacterial;
2. Time of appearance:
• early – catarrhal and rash periods;
• late – pigmentation period;
Complications – cont.3. Topics: • respiratory system (pneumonia, sinusitis,
laryngitis, laryngo-tracheitis, bronchitis, bronchiolitis, pleuritis),
• gastro-intestinal (stomatitis, enteritis, colitis), • nervous system (encephalitis,
meningoencephalitis, meningitis, psychosis), • eyes (conjunctivitis, blepharitis, keratitis, kerato-
conjunctivitis), • ears (otitis, mastoididtis), skin (pyodermia,
phlegmona), • urinary tract (pyelitis, cystitis, pyelonephritis).
Complications – encephalitis
• Rare complication• Mostly after severe cases, but can be after mild
and moderate • Mostly in adults • Mortality up to 25%• Possible consequences: paresis, intellectual
deficit, epilepsy.
Measles is proved to play role in development of slow sclerosing panencephalitis
Complications – bacterial
• Mostly under 3 years of age (→ antibiotics are indicated in most cases).
• Usually, the earlier → the more severe
• Can mask by clinical presentation of measles itself
Laboratory diagnosis
• CBC: leucopenia, lymphocytosis
• Cytology of nasal discharge – typical multinuclear giant cells
Required confirmation: • Virusology – PCR and IFA (in rash period
can already be negative)
• Serological – titer of IgM increases, IgG → measles in anamnesis
Differential diagnosis
• Catarrhal period – acute respiratory viral infections;
• Rash period – rubella, scarlet fever, enteroviral infection, pseudotuberculosis, meningococcemia, allergic exanthema, ampicillin usage at infectious mononucleosis.
Treatment
• Regime – mouth and eyes washing• Etiotropic therapy (ribavirin) – unclear efficacy,
recommended for encephalitis cases • Vitamin A • Antibiotics (children under 3 years of age,
immunodeficient or development of complications)
• Detoxication• Symptomatic
Hospitalization of all children under 2 years of age
Prophylaxis
Active
• Alive attenuated vaccine (MMR) – at 1 and 6 years.
• After vaccination, a measles-like syndrome can develop – these children are not contagious
• Urgent vaccination after contact- first 5 days
Prophylaxis
Passive
• Anti-measles Ig – first 3 days after contact
• Indications: 3 months - 2 years of age without vaccination or disease in the past; patients with chronic infections and immunosupression, pregnant.
Contagious period
Since the last days of incubation period – till 4th days (10th day in case of complications) after appearance of the rash on the skin
Isolation
• Till the 4th day after appearance of the exanthema (uncomplicated cases)
• Till the 10th day after appearance of the exanthema (complicated cases)
RUBELLA
RUBELLA
Viral infection – acquired (airborne, mild clinical presentation and benign outcome) or inborn (transplacental transmission, severe defects of the fetus)
Etiology
• Virus was isolated in 1962• RNA-containing • Togaviridae, genus Rubivirus• Virus antigenically identical all over the
world → repeated cases very rare• Virus is not stable
Epidemiology• Way of transmission – airborne • Incubational period – 9-21 days• Most cases in children of 2 to 9 years • The source of infection is a patient from 1-2
weeks of incubational period and till 3 weeks of the disease
• Inborn rubella – can be contagious up till 1-2 years
• Can be isolated from respiratory secretions, blood, urine
• Index of contagiosity is 80-90% • Most cases - in winter and spring
Inborn rubella - pathogenesis
1. Direct cytopathic action on the fetus (lens and cochlear cells)
2. Mitosis activity delay
Critical periods: • brain – 3-11 weeks of gestation, • eyes and heart – 4-7 weeks, • ears – 7-12 weeks
If rubella at 2 months – more risk of catarrhact and heart defects; in 3-4 months - CNS defects
Inborn rubella – clinics
Typical Triad:
1. Deafness
2. Blindness
3. Heart defects
Very often + Brain defects, defects of any organ and system, thrombocytopenia, developmental delay …
Clinics – periods
1.Incubational period - 9-21 day
2.Prodromal period – can be several hours; mild intoxication and catarrhal period, lymph nodes enlargement
3.Rash period - 3-4 days
4.Convalescence period
Clinics
1. Intoxication syndrome : mild; fever under 37,5 – 1-2 days
2. Exanthema syndrome
3. Lymphadenitis – posterior cervical and occipital lymph nodes
Clinics – rash characteristics
1. Character of the elements – pale macular2. Prevalent localization – face, back, buttocks, extension
surfaces of the extremities3. Background skin color – normal 4. Time of appearance – 1st day of the disease 5. Connection to fever – mild if any fever 6. Itching – no7. Fusion of rash elements – no 8. Step-wise appearance – no9. Presence of enanthema –palate enanthema
(concurrent with rash, Forscheirmer spots) 10. Disappearance of the rash – no residuals
Classification
1. Typical (mild, moderate, severe) and atypical (low-grate, asymptomatic)
2. With / without complications
3. Acquired or inborn
Complications
• Arthritis and arthralgias – 1-2 week of the disease, more often in females
• Encephalitis (1:6 000) –- on the 5-8th day
• Thrombocytopenic purpura – 1-2nd week
Complications
• Encephalitis : fever to 40, symptoms of encephalitis
• Mortality rate is 15-20%
• Recovery takes 2-3 months, can be residual symptoms
Laboratory diagnosis
• CBC: Plasmocytes (Turke cells)– to 10-20%
Required confirmation• Virusology – PCR and IFA, nasal
secretion, CSF
• Serological – increase of IgM titer Ig M – positive 12 weeks after disease;
Ig G - anamnestic
Differential diagnosis
I. Infectious causes:
•Measles, esp. mitigated
•Scarlet fever
•Exanthema subitum
•Parvoviral infection
•Varicella
•Infectious mononucleosis
•Pseudotuberculosis
•CMV
•Acute toxoplasmosis
•Enteroviral infection
•Leptospirosis
•Rosenberg erythema
•Trichinellosis
•Syphilis
Differential diagnosis
II. Not – infectious causes:
• Reaction to measles vaccine
• Allergic dermatitis
• Drug reactions
Treatment
• Uncomplicated forms - no treatment
• At arthralgias – NSAIDs
• Encephalitis and meningitis – appropriate treatment
Prophylaxis
Active • Alive attenuated vaccine (MMR) – at 1 and 12-
14 (girls) years. • Contraindicated in pregnant, 3 months before
pregnancy, immunodeficient, at hypersensitivity to amynoglicozides and yolk
Passive • In pregnant women, but low efficacy
Contagious period
Since last 1-2 weeks of incubation period – till 2-4 weeks after appearance of the disease
Isolation
• 4 first days of the disease; inborn rubella - 2 years
• At contact with pregnant woman without rubella in the past – Ab evaluation twice in dynamics
SCARLET FEVER
Scarlet fever
Acute infectious disease caused by
β-hemolytic Streptococcus Group A
and presented with
(1) intoxication syndrome,
(2) tonsillitis,
(3)exanthema
Etiology
• β-hemolytic Streptococcus, group A • Produces exotoxin (Dick’s) →
responsible for clinics (Rash)• If anti-toxic immunity is absent →
scarlet fever• If anti-toxic immunity is present →
tonsillitis, pharyngitis, carriage, etc.
Epidemiology• Antroponous infection• Source of the infection – patient with scarlet
fever, any other streptococcal infection or carriage
• Autumn – spring months, periods of every 2 years
• Mostly – children under 8-9 years of age• Main way of transmission – airborne; can
be also contact and food-borne • Easiest transmission – in close rooms
Epidemiology
• Index of contagiosity is 40%
• The patient is contagious since first clinics till 24 hours after start of antibiotic therapy
• Mild cases and carriers are more dangerous epidemically
• Due to usage of antibiotics, nowadays repeated cases are possible
Pathogenesis
• Entry of infection – tonsillar mucous, seldom – macerated or burnt skin (atypical forms) → local inflammation
• Catarrhal, lacunar or necrotic tonsillitis and toxin adsorption from the entry → intoxication and rash
Pathogenesis
3 main pathogenetic components of scarlet fever:
1. Toxic
2. Septic
3. Allergic
PathogenesisToxic component: • Intoxication syndrome (fever, vomiting,
headache)• Activated sympatic tonus (red dermographism,
tachycardia, skin dryness, elevated blood pressure)
• Rash • In severe cases - hemodynamic disturbances,
adrenal hemorrhages, brain edema
Pathogenesis
Septic component:
• Necrotic changes at the infection entry (necrotic tonsillitis)
• Septic purulent complications (sinusitis, otitis, pneumonia, purulent lymphadenitis, septic arthritis, etc.)
PathogenesisAllergic component: Sensibilization to β-hemolytic Streptococcus
and damaged tissue antigens. Appears in the 2-3rd week of the disease
• Polymorphic skin rash• Acute allergic lymphadenitis• Glomerulonephritis• Rheumatic fever, myocarditis• Sinoviitis.
Immunity Permanent non-specific antitoxic &
Transient type-specific antimicrobial ↓
Repeated cases of scarlet fever are rare (in cases of inadequate production of antitoxic
antibodies) but
any other streptococcal infections in the future possible (tonsillitis, erysipelas, otitis,
pneumonia, etc.)
Clinics
1. Incubational period - 2-7 days
2. Rash period - since 1st day: Intoxication syndrome + tonsillitis + rash
3. Recovery period
Clinics – rash characteristics1. Character of the elements – pointed macular m2. Prevalent localization – cheeks, side surfaces of the body,
flexion surfaces of the extremities, inquinal area; nasolabial triangle is pale
3. Background skin color – hyperemic 4. Time of appearance – 1st day of the disease 5. Connection to fever – intoxication of the first day 6. Itching – no7. Fusion of rash elements – no 8. Step-wise appearance – no 9. Presence of enanthema – scarlet hyperemia of tonsillitis 10.Disappearance of the rash – scaled desquamation of palms
and feet and branny desquamation of the rest of the body
SCARLET FEVER
Pathognomic skin symptoms
• Pastia’s sign – increased brownish (tiny hemorrhages) lines in the physiological folds of the body
• Filatov’s sign - Pale nasolabial triangle
Tonsillitis
• Most often – lacunar
• Bright localized hyperemia of the pharynx (“burning pharynx”)
• Strawberry tongue – forms by the 3rd day of the disease
Classification
1. Typical (mild, moderate, severe) and atypical (extratonsillar)
• Mild: fever under 38,5С, mild intoxication, rash and tonsillitis
• Moderate: fever 39С, vomiting, prominent tonsillitis and rash
• Severe: fever 40 and higher, loss of consciousness, septic and toxic complications
2. With / without complications
Complications
1. Toxic
2. Septic
3. Allergic
Toxic complications
• Hemodynamic disturbances
• Adrenal hemorrhages
• Brain edema
• Septic shock syndrome
Septic complications
• Pneumonia
• Otitis
• Sinusitis
• Necrotic tonsillitis
• Paratonsillar and retropharyngeal abscesses
• Purulent lymphadenitis
• Septic arthritis, etc.
Allergic complications
• Rheumatic fever
• Myocarditis
• Poststreptococcal glomerulonephritis
• Polymorphic skin rash
• Acute allergic lymphadenitis
• Sinoviitis
Laboratory diagnosis
• CBC: leucocytosis, neurophiles, bands, elevated ESR
• Throat culture (confirmative) – positive for β-hemolytic Streptococcus Group A
• Serological – increase of titer of antostreptolysin O, anti-DNAsa in dynamics
Differential diagnosis
• Allergic exanthema
• Pseudotuberculosis• Measles
• Enteroviral infection
• Syphilis
• Rubella
• Meningococcemia
• Ampicillin usage at infectious mononucleosis.
Treatment
• Diet – allergy free, mechanical and thermal mild (tonsillitis)
• Antibiotics !!! 1. Penicillins – 10 days 2. Macrolides – at allergy to penicillins 3. Cephalosporins – at complications
• Detoxication• Anti-histamine drugs • Symptomatic
Prophylaxis
No vaccination
All contact patients – throat Cx & treatment of the carriers
Treatment of other streptococcal diseases
Contagious period
The last 1-2 days of incubation period – till 24 hours after beginning of adequate antibiotic therapy
Isolation
• 21 days from the beginning of the disease (term of possible complication development)
• On the 21st day – urinalysis and ECG
CHICKEN POX
Chicken Pox
Acute infectious disease caused by Varicella-Zoster virus (VZV) from Herpesviridae with airborne way of transmission, with predominant involvement of epithelium and nervous system and presented with syndromes of intoxication and exanthema
Classification of Herpesviridae
1. HSV-12. HSV-23. VZV4. EBV5. CMV6. Herpesvirus – 6 type 7. Herpesvirus – 7 type 8. Herpesvirus – 8 type
History
• Before 16th century chicken pox was not differentiated from smallpox → the name of the disease
Disease forms
1. Varicella - primary infection, with virusemia
2. Herpes Zoster – reactivation of virus from neural ganglia
Etiology
• Varicella-Zoster virus (VZV)
• DNA-containing
• Carriage can be life-long
• Very unstable (15 minutes at room air)
• Very volatile and highly transmittable
Epidemiology
Almost all children become positive by 10-14 yearsSource of the infection – patient with varicella or
herpes zoster Contagiousness starts from 10th day of incubation
and ends after 5th day after the last rash appearance
Transmission is airborne, seldom – transplacentarIndex of contagiosity is 95-98%Most of the cases – pre-school children Mostly in autumn – winterRepeated cases – in 2-3%
Pathogenesis
• Entry of infection – upper respiratory mucous
• Virusemia → skin and mucus epithelium → characteristic rash
Pathogenesis of rash
1. Capillary dilation → macula2. Serous edema → papula 3. Damage of spike layer of the skin → cell
dystrophy and necrosis → intercellular fluid collection → vesicles. Basement of vesicles is infiltrated with monocytes and lymphocytes → typical hyperemia around vesicles
4. Resorption of exudate → crusts
Pathogenesis
• All the mucous membranes of the body: macula → papula → erosion. Clinical presentations – croup, dyspepsia, dysuria
• Tropism to nervous system:Vertebral ganglia → Herpes Zoster
Brain cortex → encephalitis
Cerebellum → cerebellitis
• Immunodeficiencies → visceral forms (hepatitis, pneumonitis, carditis, etc.)
Clinics – periods
1. Incubational period - 9-21 day
2. Rash period - depends on severity, from 3-4 days to 2 weeks
3. Convalescence period (“crusts period”)
Clinics – syndromes
• Intoxication syndrome – spikes of fever with every new rash elements. Fever, fatigue, malaise, irritability, vomiting
• Exanthema syndrome
Clinics – rash characteristics
1. Character of the elements – false polymorphism (macula → papula → vesicle → crust)
2. Prevalent localization – all over the body, including hair part of the head and excluding palms and feet
3. Background skin color – normal 4. Time of appearance – first days of the disease 5. Connection to fever – fever spikes with waves of new
elements appearance 6. Itching – yes7. Fusion of rash elements – no 8. Step-wise appearance – no, but repetitive waves9. Presence of enanthema – on all mucous membranes 10.Disappearance of the rash – temporal depigmentation or mild
scars after pustules or crusts removal
CHICKEN POXCHICKEN POX
CHICKEN POX
CHICKEN POX
HERPES ZOSTER
ClassificationType Severity Course
Typical Mild Without complications
Atypical:
Rudimentary Hemorrhagic Gangrenous Generalized (visceral)
Bullious
Pustulous
Moderate With complications
Severity
Severity
• Mild – subfebrile fever, scarce to moderate elements
• Moderate – fever to 390С, numerous elements, including mucous membranes
• Severe – fever up to 40, prominent rash and intoxication, neurotoxocosis and encephalitic reactions
Atypical forms
Rudimental - after receiving antibodies (Ig or plasma injection) short before disease.
Scarce rash, vesicles may be absent, no intoxication.
Atypical formsGeneralized = visceral – in immunodeficient
patients
Severe intoxication, inflammation of inner organs caused by VZV (hepatitis, pneumonitis, carditis, nephritis, etc.
High mortality. On autopsy – necrosis foci in lungs, pancreas, adrenals, thymus, spleen, etc.
Atypical forms
Hemorrhagic form – in children with hemorrhagic diseases, hemoblastoses, patients on corticosteroids or cytostatics.
Hemorrhagic vesicles, hemorrhages into mucous and inner organs.
Prognosis is serious.
Atypical forms
Gangrenous form – in children with defects of taking care of, usually with secondary infections
inflammation around hemorrhagic vesicles with development of deep growing ulcers
Complications
1. Primary (caused by VZV)
2. Secondary (caused by bacterial infections)
Primary Complications
• Encephalitis
• Meningoencephalitis
• Myelitis
• Nephritis
• Myocarditis
CNS complications
Early complications• Develop on the first days of the disease• Severe encephalitis with high mortality• Hyperthermia, seizures, loss of consciousness, focal
signs
Late complications • Develop at the “crusts” period • Do not depend on the severity of the previous
varicella • Most often – cerebellitis
Cerebellitis
• Most often form of the varicella encephalitis • Develops after several days of normal
temperature • Clinics:
cerebellar signs – ataxia, aphasia, tremor, nistagm;
can be fatigue, headache, vomiting, fever. meningeal signs not prominent or absent
• SCF normal or mild changes • Recovery during several days or weeks
Other complications • Reye syndrome • Croup• Thrombocytopenia • VZV pneumonia (adults and teenagers mostly) • Carditis • Keratitis • Arthritis• Glomerulonephritis • Nephrotic syndrome • Pancreatits • Orchitis
Secondary complications
• Cellulitis • Pustulous and
bullous forms • Phlegmona • Abscess • Impetigo
• Bullious pyodermia
• Erysipelas
• Lymphadenitis
• Stomatitis
• Conjunctivitis
• Croup (seldom)
Chicken Pox in Newborns
• If mother develops chicken pox within 5 days before delivery or few days after, the newborn will develop severe forms with generalization and high mortality rate. Incubation period in newborns is 6-16 days.
• If mother develops chicken pox earlier than 5 days before delivery, the newborn will produce mild or rudimental forms.
Laboratory diagnosis
1. In typical clinical forms, laboratory confirmation is not required
2. If the diagnosis is in doubt: • “Tzanck” smear with sensitivity of about 60%
(from a vesicle). • VZV antigen from vesicular fluid (IFA)• PCR of vesicle fluid, CSF or blood• Serology – 4-fold increase in repeated titers in10
days
Differential diagnosis
• Herpetic infection
• Impetigo
• Herangina
• Allergic rash
• Meningococcemia
• Toxic dermatitis
Treatment
Mouth washing after every meal;
Skin bathing without scratching;
Topic antiseptic paints are contraindicated unless secondary infection develops;
Corticosteroids are contraindicated, except in late encephalitis.
Treatment
Mild and moderate cases in immunocompetent children – symptomatic therapy:
• NSAIDs for fever (absolutely exclude aspirin – risk of Reye syndrome!) (avoid ibuprofen – risk of necrotizing fasciitis)
• Anti-hystaminic drugs for itching
Treatment
Indications for etiotropic therapy: • Severe course • Visceral, gemorrhagic forms• Patients with immunodeficiencies (HIV,
primary immunodeficiencies, oncology, hematologic diseases, patients on steroids or chemotherapy, after organ transplantation)
• Newborns, first two years of age children (if mother did not have chicken pox)
• Patients older than 12 years
Treatment
Eiotropic therapy – acyclovir Start within first 24 hours! IV:
10-30 mg/kg/day in 100 ml of infusion
PO: 400mg qid for 2-6 years old 800 mg qid for older children
Course 5-7 days
Polyvalent IV Immunoglobulin (0,2-0,5 ml/kg) or specific VZV Immunoglobulin
Treatment
Antibiotics – for secondary complications, gangrenous form
Encephalitis:
Acyclovir
Corticosteroids
Pathogenic treatment
Prophylaxis
Chemoprophylaxis with acyclovir is not effective
Active prophylaxis – live attenuated VZV-vaccine
Children from 1 to 13 years – once
Older children and adults twice in 4-8 weeks (after previous testing for VZV-IgG)
Prophylaxis
Passive prophylaxis : VZIG –1,25 ml/10 kg, IM, once, at first 48-96 hours of incubation. Incubation period can prolong till 28 days. VZIG circulates in the body 2-4 weeks.
Indications: 1. Immunodeficiences, 2. Pregnancy (after previous testing for VZV-IgG),3. Newborns from mothers who developed
varizella within 5 days before and 2 days after delivery.
ROSEOLA INFANTUM, sixth disease
Roseola infantum
Roseola infantum (Human Herpesviruses 6 and 7) is a mild febrile, exanthematous illness occurring almost exclusively during infancy
History
• Roseola was described as a distinct illness at the beginning of the 20th century.
• HHV-6 was discovered in 1986, 22 yr after discovery of the 5th human herpesvirus (Epstein-Barr virus), first – in peripheral blood mononuclear cells (PBMCs) of adult patients with AIDS or lymphoproliferative diseases
• HHV-7 was identified in 1990 in the peripheral blood mononuclear cells of an HIV-uninfected adult
Causative agents
• 2/3 cases - HHV-6 (=exanthem subitum, = sixth disease), also causes other diseases.
• 1/4 cases - HHV-7
• Other cases - viruses (e.g., echovirus 16)
Etiology
• ß-herpesvirus subfamily of herpesviruses (includes HHV-6, HHV-7 & CMV)
• Large double-stranded DNA genome
• Establishment of latency after primary infection
• The principal target cells for HHV-6 and HHV-7 infection in vivo are CD4 T cells
Epidemiology
• HHV-6 mostly occurs early in life: 90% of newborn are HHV-6 seropositive (maternal
antibodies) 4–6 mo of age - 0–60% are seropositive 12 mo of age - 60–90% are seropositive 3–5 yr - 80–100% are seropositive
• Peak of disease is 6–15 mo of age • > 95% of cases - in children younger than 3 yr• Transplacental antibodies are likely to protect
most infants until 6 mo of age.
Epidemiology
• Higher incidence during spring and fall months
• Contact with roseola is rearily reported
• Incubation period averages 10 days (range of 5–15 days).
• Most adults excrete HHV-6 and HHV-7 in saliva → primary sources of virus for children
• No congenital HHV-7 infections have been described
Pathogenesis
• Ports of entry - oral, nasal, or conjunctival mucosa
• HHV-6 can suppress all cellular lineages within the bone marrow
Course – periods 1st period – Intoxication syndrome: Acute beginning High fever: 37.9 to 40°C (101–106°F), average of
39°C (103°F) Irritability, anorexia Normal behavior despite high temperatures Catarrhal syndrome is not prominent
Fever persists for 3–5 days → resolves abruptly (“crisis”)
Occasionally, the fever may gradually diminish over 24–36 hours (“lysis”)
Course – periods2nd period - Exanthema period Rash appears within 12–24hr after fever resolution Rose colored, fairly distinctive. However, it may be
confused with exanthems resulting from rubella, measles, or erythema infectiosum
Begins on the trunk and usually spreads to the neck, face, and proximal extremities
Not pruritic, and no vesicles or pustules develop Lesions typically remain discrete but occasionally
may become almost confluent After 1–3 days, the rash fades Some children experience evanescent rashes that
resolve within a few hours.
Other clinical presentations
• Mild cervical or occipital lymphadenopathy• Slight or absent catarrhal syndrome • Seizures (5–10%) at the peak of fever • Infrequently: rhinorrhea, sore throat,
abdominal pain, vomiting, and diarrhea• In Asian countries, ulcers at the
uvulopalatoglossal junction (Nagayama spots) are common in infants with roseola
HHV-7 clinical particularities
• Slightly older age
• Lower mean temperature
• Shorter duration of fever
CNS involvement
• Febrile seizures
• Fontanelle bulging
• Encephalitis and meningoencephalitis (6% of children with unknown-cause encephalitis were CSF positive for HHV-6)
Other clinical forms• Chronic fatigue syndrome • Nonspecific febrile disease• Hepatitis • Mononucleosis-like disease • Hemophagocytic syndrome• Intussusception• Idiopathic thrombocytopenic purpura• Recurrent aphthous stomatitis• Myocarditis• Disseminated disease
Clinical forms in immunocompromised patients
• Encephalitis
• Pneumonitis
Other probable etiologic role
• Multiple sclerosis
• Various malignancies (non-Hodgkin lymphoma, Hodgkin disease, cervical and oral carcinoma, and leukemia)
Diagnosis
• Serology: HHV-6 immunoglobulin (Ig) M and 4-fold increases or decreases in HHV-6 or -7 IgG antibodies
• Virus culture: incubation of PBMCs for days to several weeks (only in research laboratories)
• PCR: serum or cerebrospinal fluid (in PBMCs, saliva, and tissues – can be latent infection)
• In situ hybridization and immunohistochemistry
Laboratory findings
• WBC counts of 8,000–9,000/µL - first few days
• 4,000–6,000/µL - by the time of exanthema
• Relative lymphocytosis (70–90%)
• CSF is normal
• In meningoencephalitis and encephalitis - mild pleocytosis, predominance of mononuclear cells, normal glucose, and normal to slightly elevated protein
Differential doagnosis
After rash appearance:
• Rubella (most similar)
• Measles
• Enteroviruses
• Scarlet fever
• Drug hypersensitivity
Treatment
Etiologic treatment • HHV-6 - ganciclovir, cidofovir, and foscarnet
(but not acyclovir) • HHV-7 - cidofovir and foscarnet Questionable clinical effect !
Indications for etiologic treatment: • Children with neurologic complications • Immunocompromised children
Treatment
Supportive therapy
• Acetaminophen or ibuprofen
• Adequate fluid balance
Prognosis
• Great majority - excellent, no sequelae
• Encephalitis, hepatitis, pneumonitis, disseminated disease, or hemophagocytosis syndrome – high mortality
Prevention
• Healthy immune carriers with latent viral infections transmit infection to susceptible infants and children
• No specific recommendations for prevention
• No vaccine has been developed
PARVOVIRAL INFECTION (INFECTIOUS ERYTHEMA, «fifth disease»)
Parvovirus B19
ERYTHEMA INFECTIOSUM (FIFTH DISEASE) is a benign, self-limited exanthematous illness of childhood
History
1983: Anderson and colleagues identified Parvovirus B19 as the cause of erythema infectiosum or fifth disease
Etiology
• Genus Erythrovirus, family Parvoviridae
• Small DNA viruses
• Relatively heat- and solvent-resistant
Epidemiology
• Common and worldwide• 70% of cases occur between 5 and 15 yr of
age• Seasonal peaks: late winter and spring• Seroprevalence: 40–60% of adults are positive • Transmission: respiratory route • Transmission rate in households: 15 to 30% • Also transmitted by blood products
Pathogenesis
• Primary target - erythroid cell line: lysis of these cells → progressive depletion & transient arrest of erythropoiesis
• No apparent effect on the myeloid cell line
• Erythrocyte P blood group antigen serves as a cellular receptor for the virus
• Also possess this antigen: endothelial cells, placenta, and fetal myocardial cells
Course
Biphasic illness
1st phase: 7 to 11 days after inoculation: • Viremia and nasopharyngeal viral shedding • Fever, malaise, and rhinorrhea• Reticulocyte counts dropping to undetectable levels• Mild, clinically insignificant anemia
But: Individuals with chronic hemolytic anemia often require transfusion
Appearance of specific antibodies causes resolution of symptoms and anemia
Course
2nd phase: 17–18 days after inoculation: • Rash, arthralgia (due to postinfectious immune
response) and possible lymphadenopathy • In impaired humoral immunity → chronic red cell aplasia,
neutropenia, thrombocytopenia, and marrow failure• In children on chemotherapy for leukemia, those with
congenital immunodeficiency states, transplant patients, and those with AIDS are at risk for chronic B19 infections
• In fetus: hydrops and stillbirth, but no teratogenicity (erythroblasts and extramedullary hematopoiesis damage)
Forms of infection
• Many infections - clinically inapparent
• Children - erythema infectiosum
• Adults - acute arthropathy with or without the rash of erythema infectiosum
Clinics - Erythema infectiosum
1. Incubation period: 4 to 28 days (average, 16–17 days)
2. Prodromal phase: – Low-grade fever– Headache – Mild upper respiratory tract infection
Clinics - Erythema infectiosum
3. Exanthema period:
Three stages:
1. Erythematous facial flushing - “slapped-cheek”
2. Spread to trunk and proximal extremities - diffuse macular erythema
3. Central clearing of macular lesions → lacy, reticulated appearance
Clinics – rash characteristics
1. Character of the elements – previous slide 2. Prevalent localization – more prominent on extensor
surfaces, but palms and soles are spared3. Background skin color – normal 4. Time of appearance – 3rd – 4th day of the disease5. Connection to fever – afebrile and not ill-appearing 6. Itching – mild in older children and adults7. Fusion of rash elements – no 8. Step-wise appearance – previous slide 9. Presence of enanthema – palate 10.Disappearance of the rash – spontaneous, no
desquamation
PARVOVIRAL INFECTION (INFECTIOUS ERYTHEMA, «fifth disease»)
Rash characteristics
• The rash tends to wax and wane over 1–3 wk, can recur with exposure to sunlight, heat, exercise, and stress
• Atypical papular, purpuric, vesicular rashes are also described
Other clinical forms
• Arthritis and arthralgia
• Transient aplastic crisis
• In immunocompromised persons
• Fetal infection
• Myocarditis
• Other cutaneous manifestations
Arthritis and arthralgia
Can be: 1. Complication of fifth disease2. The only clinical manifestation of B19 infection
• Much more common in adults and older adolescents• Females > males• Range from diffuse arthralgias with morning stiffness
to frank arthritis• The joints most often affected: hands, wrists, knees,
and ankles, etc.• Self-limited within 2–4 wk
Transient aplastic crisis
Develops in patients with sickle cell disease, thalassemia, hereditary spherocytosis, and pyruvate kinase deficiency
Incubation period is shorter: coincident with the viremia
Fever, malaise, and lethargySigns and symptoms of profound anemia (pallor,
tachycardia, and tachypnea)Rash is rarely presentConcurrent vaso-occlusive pain crisis
Immunocompromised persons
Chronic infections: Chronic anemia, neutropenia, thrombocytopenia, or complete marrow suppression
Fetal infection
• Nonimmune fetal hydrops and intrauterine fetal demise <5%
• Viral-induced red cell aplasia
• B19 has not been associated with other birth defects
Myocarditis
• In fetuses, infants, children and a few adults
• Outcomes: from complete recovery to chronic cardiomyopathy to fatal cardiac arrest
Other cutaneous manifestations
Papular-purpuric “gloves and socks” syndrome (PPGSS) is distinctly associated with B19 infection:
PruritusPainful edemaErythema of distal extremities (glove and sock
distribution) Acral petechiaeOral lesionsSelf-limited within a few weeks
Diagnosis
• Serologic tests
• Determination of anti-B19 IgM
• Seroconversion of anti-B19 IgG in paired sera
• The virus cannot be isolated by culture
• PCR or nucleic acid hybridization
Differential diagnosis
• Rubella
• Measles
• Enteroviral infections
• Drug reactions
• Juvenile rheumatoid arthritis
• Systemic lupus erythematosus
• Other connective tissue disorders
Treatment
• No specific antiviral therapy
• Anemia and bone marrow failure in immunocompromised children - intravenous immunoglobulin (IVIG)
• Fetuses with anemia and hydrops - intrauterine transfusions
Complications
• Arthralgias or arthritis in adolescents and adults (may persist after resolution of the rash)
• Thrombocytopenic purpura
• Aseptic meningitis
• Hemophagocytic syndrome
Prevention
Children with erythema infectiosum are not infectious at presentation because the
rash and arthropathy represent immune-mediated, postinfectious phenomena
↓
Isolation and exclusion from school or childcare are unnecessary and ineffective
after diagnosis
Prevention
Children with B19-induced red cell aplasia (transient aplastic crisis) are infectious when they present and demonstrate a
more intense viremia
↓
Isolation for at least 1 wk and until after resolution of fever
Prevention
• There are no data to support the use of IVIG for postexposure prophylaxis in pregnant caregivers or immunocompromised children
• No vaccine is available
KAWASAKI DISEASE
KAWASAKI DISEASE
Kawasaki Disease
Mucocutaneous lymph node syndrome = infantile polyarteritis nodosa
Acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967.
Importance
• The disorder occurs worldwide, with Asians at highest risk
• Approximately 20% of untreated patients develop coronary artery abnormalities including aneurysms, with the potential for the development of coronary artery thrombosis or stenosis, myocardial infarction, aneurysm rupture, and sudden death
• Kawasaki disease has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in the United States and Japan.
Etiology
Causative agent is still unknown
Features to support an infectious origin:
• Age group affected• Occurrence of periodic epidemics• Wavelike geographic spread of illness during the
epidemic• Self-limited nature of the illness• Clinical features of fever, rash, enanthem,
conjunctival injection, and cervical lymphadenopathy• Probable genetic predisposition • Probable passive maternal antibodies → seldom in
kids < than 4 mo• Absence in adults → widespread immunity
Epidemiology
• 3,000 cases are diagnosed annually in the United States
• The incidence of Kawasaki disease in Asian children is substantially higher than in other racial groups, but the illness occurs worldwide in all ethnic groups
• In Japan, more than 170,000 cases have been reported since the 1960s
Epidemiology
• Kawasaki disease is not a new illness; autopsy reports of infantile periarteritis nodosa before the 1960s appear in retrospect to have represented fatal Kawasaki disease.
• The illness occurs predominantly in young children; 80% of patients are younger than 5 yr, and only occasionally are teenagers and adults affected.
Pathogenesis
1. Severe vasculitis of all blood vessels, mostly medium-sized arteries (predilection for coronary arteries)
2. Edema & inflammatory infiltration of vascular wall (PMN, macrophages, lymphocytes and plasma cells)
3. In severe cases - all three layers are involved
Pathogenesis of vasculitis
Destruction of the internal elastic lamina↓
Lost of structural integrity and weakens ↓
Dilatation or aneurysm formation↓
Formation of thrombi ↓
Obstruction of blood flow
Pathogenesis of vasculitis
Healing phase:
Fibrosis: intimal proliferation
↓
Stenotic occlusion of the vessel
Clinical presentation • Fever 5 days and more • Bilateral conjunctivitis (without exudates) • Lips and mouth changes:
– Dryness, erythema and fissures of the lips– Strawberry tongue – Diffuse erythema of mouth and throat mucous
• Changes of hands and feet:– Erythema of hands and feet hands and feet – Indurative edema of erythema – Membranous desquamation, starting around the nails
• Polymorphous rash, mostly on the trunk • Acute non-purulent cervical lymphadenitis • Exclusion of other diseases
Particularities of fever
• High spiking (to 104°F or higher)
• Remittent
• Unresponsive to antibiotics
• Duration is 1–2 wk to 3–4 wk (without treatment)
• Prolonged fever → risk of coronary artery disease
Particularities of rash
• Rash of various forms (maculopapular, erythema multiforme, or scarlatiniform) with accentuation in the groin area
• Perineal desquamation (in acute phase)
• Periungual desquamation of the fingers and toes (1–3 wk after)
Other clinical presentation
Cardiological Pancarditis in acute phase Coronary arteries anomalies Heart failure
Neurological Extreme irritability Aseptic meningitis
Other clinical presentation
Joints Arthritis Arthralgia
Gastro-intestinal
Diarrhea, vomiting, pain Mild hepatitisHydrops of the gallbladder
Other clinical presentation
Respiratory Respiratory syndrome Otitis media Lung infiltrates
Others UrethritisSterile pyuria Edema of testes Periphery gangrene Hearing disturbances Aneurisms of non-coronary vessels of medium size
Particularities of arthritis
• More common in girls
• May occur early in the illness
• May develop during the 2nd–3rd week
• Generally affects hands, knees, ankles, or hips
Particularities of cardiac involvement
• Most important manifestation of Kawasaki disease
• Myocarditis (tachycardia and ↓ ventricular function) - 50% of patients
• Pericarditis (small pericardial effusion) - common during the acute phase
Particularities of coronary artery aneurysms
Up to 25% of untreated patients• During the 2nd–3rd wk of illness• Can be detected by two-dimensional
echocardiography • Giant coronary artery aneurysms (≥8mm
internal diameter) → greatest risk for rupture, thrombosis or stenosis, and myocardial infarction
__________________________________Valvular regurgitation and systemic artery
aneurysms may occur but are uncommon.
Course of Kawasaki disease
Three clinical phases:
1. Acute febrile phase
2. Subacute phase
3. Convalescent phase begins when all
Acute febrile phase
• 1–2 wk of duration
• Fever and the other acute signs of illness
Subacute phase
• Begins when fever and other acute signs have abated - lasts until about the 4th wk
• Irritability, anorexia, and conjunctival injection may persist
• Desquamation, thrombocytosis, the development of coronary aneurysms, and the highest risk of sudden death
Convalescent phase
• Begins when all clinical signs of illness have disappeared and continues until the erythrocyte sedimentation rate (ESR) returns to normal, approximately 6–8 wk after the onset of illness
Predictors of severe outcome• Male gender• Age < 1 yr• Prolonged fever• Recurrent fever after an afebrile period• Following laboratory values at presentation:• Low hemoglobin level• Low platelet level • High neutrophil and band counts• Low albumin level • Low age-adjusted serum IgG level
Diagnostic Criteria for Kawasaki Disease
• Presence of fever for at least 5 days + at least four of five of the other characteristic clinical features of illness
Diagnostic Criteria for Kawasaki Disease
1. Bilateral bulbar conjunctival injection, generally nonpurulent
2. Changes in the mucosa of the oropharynx, including injected pharynx, injected and/or dry fissured lips, strawberry tongue
3. Changes of the peripheral extremities, such as edema and/or erythema of the hands or feet in the acute phase; or periungual desquamation in the subacute phase
4. Rash, primarily truncal; polymorphous but nonvesicular5. Cervical adenopathy, =1.5cm, usually unilateral
lymphadenopathy6. Illness not explained by other known disease process
Diagnosis
Atypical or incomplete cases in which a patient has fever with fewer than four other features of the illness and then develops coronary artery disease have been described worldwide. Incomplete cases are most frequent in infants, who unfortunately have the highest likelihood of developing coronary artery disease.
Recognition depends on a high index of suspicion and knowledge of the characteristic clinical features of the illness.
Differential diagnosis
• Scarlet fever• Toxic shock syndrome• Measles• Drug hypersensitivity reactions• Stevens-Johnson syndrome• Juvenile rheumatoid arthritis• Rocky Mountain spotted fever• Leptospirosis
Laboratory Findings
No specific diagnostic test for Kawasaki disease exists
Characteristic laboratory findings• White blood cell count is normal to elevated• Predominance of neutrophils and immature forms• Elevated ESR, C-reactive protein and other acute
phase reactants• Normocytic anemia• Platelet count: normal in the 1st wk and rapidly
increases by the 2nd–3rd wk, sometimes exceeding 1,000,000/mm3
Other laboratory findings
• Antinuclear antibody and rheumatoid factor are negative
• Sterile pyuria
• Mild elevations of the hepatic transaminases
• Cerebrospinal fluid pleocytosis
Frequency of Laboratory Findings
Finding Frequency Comments
Leukocytosis 100% Till 15*10*9, Bands increased
Anemia 50-90% Normochromic
Hypoalbuminemia 10-50%
Frequency of Laboratory Findings
Finding Frequency Comments
Increased acute phase proteins
>90% ESR usually >35mm/h
Sterile pyuria 50-90% Can be intermittent
Proteinuria <10%
Frequency of Laboratory Findings
Finding Frequency Comments
Transaminases elevation
50%
Bilirubin elevation
<10%
Arthritis 33% without treatment, 8% after Ig injection
Cytosis till 25 000/mm3, neutrophils predominate in 80% of patients
Frequency of Laboratory Findings
Finding Frequency Comments
CSF cytosis 40% ≈ 20/mm3, 90% neutrophils, 10% mononuclears, protein and glucose are normal
Thrombocytosis >90% In subacute phase, 500 000 till 2 000 000/mm3
Two-dimensional echocardiography
Monitoring of coronary artery abnormalities:
Terms of performance:
1. At diagnosis
2. After 2–3 wk of illness
3. At 6–8 wk after onset of illness If normal → optional follow-up If abnormal → frequent echocardiographies
and potentially angiography
Treatment
ACUTE STAGE
• Intravenous immunoglobulin 2g/kg over 10–12hr
• Aspirin 80–100mg/kg/24hr divided every 6hr orally until 14th illness day
Treatment
CONVALESCENT STAGE
• Aspirin 3–5mg/kg once daily orally until 6–8 wk after illness onset (till ESR normalization)
Treatment
LONG-TERM THERAPY FOR THOSE WITH CORONARY ABNORMALITIES
• Aspirin 3–5mg/kg once daily orally ± dipyridamole 4–6mg/kg/24hr divided in two or three doses orally (most experts add warfarin for those patients at particularly high risk of thrombosis)
Treatment
ACUTE CORONARY THROMBOSIS
• Prompt fibrinolytic therapy with tissue plasminogen activator, streptokinase, or urokinase under supervision of a pediatric cardiologist
Comments
Patients receiving long-term aspirin therapy are candidates for influenza and VZV vaccines to reduce the risk of Reye syndrome
Intravenous immunoglobulin (IVIG) treatment
• Rapid defervescence and resolution of clinical signs
• Reduce of coronary disease risk from 20–25% in children treated with aspirin alone to 2–4% in those treated with IVIG and aspirin
• Should be administered within the first 10 days of illness
Treatment of non-responders
Non-responders to initial IVIG infusion
↓
Additional infusion of IVIG, 2g/kg
↓
Non-responders to initial IVIG infusion
↓
Cautious use of corticosteroids
Patients with aneurysms
Evaluation: echocardiography stress testing possibly angiographyTreatment: Catheter intervention with percutaneous
transluminal coronary rotational ablation Directional coronary atherectomy Stent implantation
Prognosis
• Depends on coronary disease severity• In Japan, fatality rates are now < 0.1%• 50% of coronary artery aneurysms resolve
within 1–2 yr after illness• Giant aneurysms are unlikely to resolve
and are most likely to lead to thrombosis or stenosis
• Coronary artery bypass grafting may be required
We are done!!!
top related