Staphylococcal Scalded Skin Syndrome On Infant: Case Report Infection Division

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CASE REPORT

INFECTION DIVISION

STAPHYLOCOCCAL SCALDED SKIN SYNDROME ON INFANT

Dewy Ratyh Ibrahim

Department of Pediatrics, Faculty of Medicine, Hasanuddin University


RSUP Dr. Wahidin Sudirohusodo, Makassar

Introduction

Staphylococcal scalded skin syndrome (SSSS), also known as Ritter von

Ritterschein disease in newborns or Ritter's disease or staphylococcal epidermal

necrolysis or bullous pemphigoid is a skin disorder caused by staphylococcal

exotoxins.1 Staphylococcal Scalded Skin Syndrome (SSSS) is a skin disorder

characterized by generalized exanthem, extensive blistering with erosions and

superficial desquamation. This disorder is caused by exfoliative toxins (ETs), namely

exfoliative toxins A (ETA) and B (ETB) produced by strains of Staphylococcus aureus

(usually phaga group 2)2,3.

The incidence of SSSS is most common in children less than 5 years old,

neonates 3-15 days old, and in immunocompromised adults. 4 This syndrome has a

clinical picture ranging from erythematous macular form followed by blistering and/or

diffuse exfoliation in a limited to large area of the skin. The area of skin that is often

involved is the flexural area, while the mucous membranes are not involved. 3

Management of SSSS is in the form of systemic antibiotics so that hospitalization,

1
emolient administration, monitoring of fluid and electrolyte balance, analgesics and

topical antibiotics are required.

CASE REPORT

Patient Identity

Name : Child. E.R


Gender : Perempuan
Birthdate : 07/08/2019
Age when chosen as case : 5 bulan 4 hari
Address : Toraja
Admission date : 11/01/2020
Medical record number : 907279
Date when chosen as case : 11/01/2020

PARENTS IDENTITY

Father Mother
Name Mr. M Mrs. P
Age 32 years old 30 years old
Education Diploma High School
Occupation Enterpreneur Maid

I. HISTORY TAKING (Subjective)

Based on alloanamnesis with the subject’s parents.

Chief complaint : Blisters on skin folds.

1. Present Medical History

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The patient was referred from a pediatrician practice with a diagnosis of Suspect

Steven Johnson Syndrome. There were skin blisters noticed in the last 2 months

before admission to hospital. Initially, in the form of reddish spots filled with fluid on

the arms then spread to the folds of the body, to be precise in the neck, nape,

hands, feet and groin. In the past 2 months before being admitted to the hospital, the

blisters appeared as bubbles filled with yellow fluid, then burst and came back. No

fever, no seizures, no cough, no spasms, no vomiting. The child wants to drink milk.

Urinate normally, yellow urine. Regular bowel movements, yellow feces.

2. Past Medical History

History of treatment in a doctor's office with the same complaint and received

antibiotic therapy (Cefadroxil), dexamethasone and paracetamol but there was no

improvement. The history of being given drugs that was applied is disputed. Allergy

history does not exist.

3. Family Medical History

Both parents are in good health. There is no family history of allergic disease.

4. Social History

 Maternal Pregnancy History:

The patient is firstborn, the mother's pregnancy at that time is a desired

pregnancy. During pregnancy, the mother routinely controls pregnancy at an

obstetrician's practice every month and gets vitamins and blood booster tablets.

There is no history of illness during pregnancy. History of drug consumption, face

creams and herbs is denied. History of vaginal discharge is denied.

 Birth History

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The baby was born by C-section on the indication of the baby's breech position,

and the baby was born with the help of a doctor. Term pregnancy, cried

immediately, and did not appear blue. Birth weight 3200 gr, birth length 49 cm.

After being treated for three days, the mother and baby were allowed to go

home.

 Post Birth History:

The patient received an injection of vitamin K and Hepatitis B after birth. The

condition of the patient after birth according to the mother was good, the patient

was never jaundice, never had any seizure, and had active movements.

 Nutrition Status:

Patient receives breast milk from birth to present date, and the child has not

been given complementary foods yet.

 Immunization Status:

Immunizations were started immediately after birth, the patient had received

basic immunization with BCG 1 time, Hepatitis B 1 time, Pentabio 3 times and

Polio 4 times.

 Basic Child Care Status

o Nurture (physical-biomedical)

The mother pays attention to the food consumed during pregnancy and after

birth, the baby is still breastfed. Basic health care is also provided by the

mother by giving the child immunizations. If the patient is sick, the patient is

taken to a doctor's office near the patient's house. The child get clean and

comfortable clothes to wear. The patient lives in a residential area, and in a

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house which is inhabited by 4 people, the house is relatively spacious with

adequate ventilation and is maintained regularly.

o Love (psychosocial)

Patient gets enough affection from father and mother. The child is born from

the first marriage of both parents and is an expected child. Affection is given

in the form of attention up to the handling of the children's diseases.

o Hone (stimuli)

The patient's mother pays full attention to the child's development.

II. PATIENT’S DATE WHEN CHOSEN AS CASE (11 January 2020)

History Taking (Subjective)

The patient was admitted as a case at 5 months 4 days old. The patient was

referred from a pediatrician practice with a diagnosis of Suspect Steven Johnson

Syndrome. There were skin blisters noticed in the last 2 months before admission to

hospital. Initially in the form of reddish spots filled with fluid on the arms then spread to

the folds of the body, to be precise in the neck, nape, hands, feet and groin. In the past

2 months before being admitted to the hospital, the blisters appeared as bubbles filled

with yellow fluid, then burst and came back. No fever, no seizures, no cough, no

spasms, no vomiting. The child still wants to drink milk. Urinate regularly, yellow urine.

Regular bowel movements, yellow feces.

Physical Examination (Objective)

A. Present Status

General Condition : Good

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Consciousness : GCS 15 (E4M6V5)

Blood Pressure : 90/50 mmHg

Pulse : 98 times/minute

Respiratory Rate : 30 times/minute

Temperature : 36,5 ⁰C

Pain Scale : 0 FLACC

Oxygen Saturation : 99%

B. General Status

Physical Exam per System

System Description
Head Mesocephal, normocephal, large fontanel hasn’t
closed yet.
Hair Straight black, hard to pull
Face Left and right side symmetrical, no dismorphic
Eyes Pale conjunctiva, sclera not icteric, pupil isochor,
diameter 2.5 mm/2.5 mm, light reflex + / +
normal impression
Nose No visible nasal distress, nasal septum in the
middle, no visible secretions
Ears No secretions, timpanic membrane is intact
Mouth Lips are not cyanotic, tonsils size T1-T1, not
hyperemic. Pharynx not hiperemic.
Neck There was no neck stiffness, no suprasternal
retraction, no lymph node enlargement
Chest Symmetrical shape and movement, no
xylophone ribs
Lungs Symmetrical movements, sonor percussion,

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bronchovesicular breath sounds in both lung
fields, additional breath sounds are not heard
Heart The ictus cordis is not visible, the heart sounds
one and two are pure, the rhythm is regular, no
additional noise
Abdomen Flat with breathing, bowel sounds are normal,
the liver and spleen are not palpable. Abdominal
masses are not palpable.
Extremities Distal parts feel warm, capillary refill time is less
than 3 seconds, no edema, there is a BCG scar,
motor strength and tone are within normal limits,
physiological reflexes are normal, no
pathological reflexes were found.
Genital There was no visible abnormality.
Lymph Nodes No palpable enlargement
Others There are bullae on both ankles region, and

both hands (with reddish base and crustae

surrounding the bullae). There is an

erythema macula with squamous and

crustaes on neck folds, nape, arm folds, leg

folds, and also groin.

C. Anthropometry Status

Body Weight (BW) : 10 kg

Body Length (BL) : 68 cm

Head Circumference : 43,5 cm (Normocephaly based on Nellhaus curve)

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o BW/BL : Between +2 SD and +3 SD lines (overweight)

o BW/Age : On +3 SD line (overweight)

o BL/Age : Between median line and +2 SD line (normal height)

Assessment of nutritional status based on the 2011 WHO curve, the patient is

included in the criteria for overweight and normal height.

Table 1. Laboratory Resulf of RS. Wahidin Sudirohusodo

Parameter 10/01/2020 Normal Value


HB 12,7 11-16 g/dl
MCV 80 80-100 µm3
MCH 27 27-32 pg
MCHC 34 32-36 gr/dl
HCT 37 37-47%
Leukocyte 9800 4000-10.000 mm3
Erythrocyte 4.640.000 3.800.000-5.800.000/mm3
Thrombocyte 602.000 150.000-400.000/mm3
Sodium 136 136-145 mmol/L
Potassium 4,8 3,5-5,1 mmol/L
Chloride 107 97-111 mmol/L
Ureum 10 10-50 mg/dl
Creatinine 0,38 L(<1,3), P (<1,1)
SGOT 68 <38 U/L
SGPT 51 <41 U/L

Table 2. Urinalysis at RS. Wahidin Sudirohusodo

Parameter 12/01/2020 Normal Value


Color Yellow Light Yellow
PH 6,5 4,5-8,0
Specific Weight <= 1,005 1,005-1,035
Protein Negative Negative
Glucose Negative Negative
Bilirubin Negative Negative

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Urobilinogen Normal Normal
Ketone Negative Negative
Nitrite Negative Negative
Blood Negative Negative
Leukocyte Negative Negative
Vit. C Negative Negative
Leukocyte Sediment 0 < 5 lpb
Erythrocyte Sediment 1 < 5 lpb
Torak Sediment 1
Crystal Sediment -
Epithelial Cell Sediment 1
Other Sediment Bacteria 29

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III. SUMMARY

A girl aged 5 months and 4 days old was referred from a pediatrician practice

with a diagnosis of Suspect Steven Johnson Syndrome. There were skin blisters

noticed in the last 2 months before admission to hospital. Initially in the form of

reddish spots filled with fluid on the arms then spread to the folds of the body, to be

precise in the neck, nape, hands, feet and groin. In the past 2 months before being

admitted to the hospital, the blisters appeared as bubbles filled with yellow fluid, then

burst and came back. The child does not have a fever, does not have seizures, does

not cough, does not have difficulty breathing, and does not vomit. The child wants to

drink milk. Urinate regularly, yellow urine. Regular bowel movements, yellow feces.

On physical examination, the patient's condition was good, GCS 15. Vital signs were

within normal limits. Anthropometric examination showed overweight and normal

height. Lungs, heart and abdomen within normal limits. Laboratory examination

found reactive thrombocytosis. When hospitalized, parents were educated about the

course of the disease, complications and management that will be given to the

patient. While treated, the patient was consulted to the Dermatology and

Venereology Division, but a tissue biopsy was not examined because it had

improved after treatment.

IV. Diagnosis

- Staphylococcal Scalded Skin Syndrome

- Overweight

V. MANAGEMENT STRATEGY (Planning)

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a. Plans for Diagnostic

Peripheral Blood Smear, Procalcitonin, CRP, CD4, and tissue biopsy.

b. Plans for Medicine

- Connecta

- Antibiotic : Ampicillin 100 mg/kgbw/day = 250 mg/6 hours/intravenous

- Consult to dermatovenerology division : Compress NaCl 0,9% twice a day

(morning and evening) and 2 hours after that, rub Mupirocin Cream on lesions

c. Plans for Nutrition

Nutritional assessment : overweight

RDA x BBI = 110 x 7,8 = 858 kcal

Energy = 858 kcal

Protein = 32 grams

d. Plans for Observation

- Observe general condition and vital signs

- Observe disease progression, complications, and treatment responseP.

e. Providing Information, Communication, and Education

- Provide an explanation to parents about the patient's condition including

causes, disease progression, complications, prognosis and further action plans.

- Explain the importance of cooperation and support from family so that the

treatment process can run smoothly.

Further Observation

Day – 3

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Subjective: the patient's skin still looks flaky and reddish. No fever, no cough. The baby

breast feeding normally. No problem urinating, yellow urine, regular bowel movements,

yellow feces.

Objective: General condition: good. Vital signs are within normal limits. On the skin,

erythematous macules appeared accompanied by squamous and crusts in the folds of

the neck, feet, hands and groin, some of the crusts are blackish. It looks like the bullae

are starting to dry out, but new bullae appear on the legs.

Diagnosis : Staphylococcal scalded skin syndrome

Treatment :

- Connecta aff (Phlebitis)

- Antibiotic: Ampicillin IV changed into Oral Amoxicillin with dose: 15 mg/kgBW/8

hours = 150 mg/8 hours/oral

- Compress NaCl 0,9% every morning and evening for 2 hours, followed by

Mupirocin Cream rubbing on lesions after 2 hours.

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Further Observation

Day - 7

Subjective: the patient's skin appears to be mostly dry, redness has been greatly

reduced. No fever, no cough. The baby breast feeding normally. No problem urinating,

yellow urine, regular bowel movements, yellow feces.

Objective: General condition: good. Vital signs are within normal limits. On the skin,

there are erythematous macules on neck fold, legs, hands, and groin. No new bullae

appearance.

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Table 3. Laboratory Result RS. Wahidin Sudirohusodo (Kontrol)

Parameter 16/01/2020 Normal Value


HB 14,3 11-16 g/dl
MCV 82 80-100 µm3
MCH 27 27-32 pg
MCHC 33 32-36 gr/dl
HCT 43 37-47%
Leukocyte 12.400 4000-10.000 mm3
Erythrocyte 5.220.000 3.800.000-5.800.000/mm3
Thrombocyte 681.000 150.000-400.000/mm3
CD4 2147 470 – 1298 /mm3

Diagnosis : Staphylococcal scalded skin syndrome

Therapy:

- Breastmilk on demand

- Antibiotic : Amoxicillin 15 mg/kgBW/8 hours = 150 mg/8 hours/oral

- Compress NaCl 0,9% every morning and evening for 2 hours, followed by

application of concocted cream from DermatoVenerology division (Dermovate

Cream 0.05%+Fuladic Cream 2grams), and stop Mupirocin Cream.

- Patient is discharged, and need to control to the outpatient department (poly

clinic)

Further Observation

Day – 10 (Outpatient Department)

Subjective: the skin are not peeling off anymore. No fever. No breathing difficulty. No

vomiting. Regular bowel movement, soft brownish stool. Normal urination, yellowish

color.

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Objective: general condition active. Vital signs are within normal limits. There were

scars on the neck, feet, hands and groin.

Diagnosis : Staphylococcal scalded skin syndrome

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Terapi :

- Breastmilk on demand

- Antibiotic : Amoxicillin 15 mg/kgBW/8 hours = 150 mg/8 hours/oral (prepare

antibiotic for 14 days)

- Compress NaCl 0,9% every morning and evening for 2 hours, followed by

application of concocted cream from Dermato-Venerology division (Dermovate

Cream 0.05%+Fuladic Cream 2grams)

VI. Prognosis

- Qua ad vitam : dubia ad bonam.

- Qua ad functionam : dubia.ad bonam

- Qua ad sanationem : dubia.ad bonam

VII. Case Analysis

History

Staphylococcal scalded skin syndrome (SSSS) was first reported by Ritter von

Rittershain in 1956 and is known as Ritter von Rittershain disease and is often

abbreviated as Ritter's disease; synonymous with exfoliative dermatitis. At that time

SSSS was not yet known. Then Lyell in 1956 categorized it into toxic epidermal

necrolysis (TEN). It was only in 1970 through research by Milish and Glasgow with a

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mouse model and various clinical and histopathological studies that this syndrome

became an explanation and it turned out to be different from TEN.8

Epidemiology

SSSS is a very rare disease with an incidence rate of 0.09 - 0.56 cases /

1,000,000 people.4 SSSS incidence is most common in children less than 5 years,
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neonates aged 3-15 days old, and in immunocompromised adults. SSSS mortality
5
reaches 4 - 10 % even with proper antibiotic management.

Pathogenesis

This syndrome is caused by exfoliative toxins (ETs) produced by group A phaga

2 Staphylococcus aureus. These exfoliative toxins are exfoliative toxin A (ETA) and

exfoliative toxin B (ETB).

Infection is caused by S.aureus group A phaga 2 which produces ETA and ETB.

This exfoliative toxin is epidermolytic, spreads through the circulation, and acts on

desmoglein-1, which is a protein in the superficial epidermis with the function of

maintaining epithelial integrity. The toxin causes the breakdown of desmoglein resulting

in epidermolysis between the stratum spinosum and granulosum, as a result, forming

soft, thin-walled blisters that break easily with a positive Nikolsky sign. 3

Diagnosis

S. aureus infection originates in certain locations such as the skin, throat, nose,

mouth, or digestive tract. SSSS presents as reddish patches followed by complete

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peeling of the epidermis. SSSS usually begins with fever, malaise, restlessness, and

pain. Then followed by widespread redness of the skin which usually occurs in folds,

such as the neck, axilla, groin and face. Within 24-48 hours, lumps filled with fluid form,

they break easily, and leave a burning appearance. Two to three days the top layer of

the skin will shrink and there is peeling of the skin sheet, leaving an open wound that is

moist, red and painful. Open wounds will then dry up and desquamate, this condition

usually heals in 7-14 days. 11

The diagnosis of SSSS can usually be confirmed by clinical findings. This

depends on the condition of the condition characteristic of this syndrome. S.aureus

infection originates in certain locations such as the skin, throat, nose, mouth, or

digestive tract. SSSS presents as reddish patches followed by complete peeling of the

epidermis. SSSS usually begins with fever, malaise, restlessness, and pain. Then

followed by widespread redness of the skin that commonly occurs in folds, such as the

neck, axilla, groin and face without involving the mucosa with a positive Nikolsky sign.

Within 24-48 hours, lumps filled with fluid form, they break easily, and leave a burning

appearance. Two to three days the top layer of the skin will shrink and there is peeling

of the skin sheet, leaving an open wound that is moist, red and painful. Open wounds
10,11,12
will then dry up and desquamate, this condition usually heals in 7-14 days.

In this case, the baby was 5 months old. SSSS generally occurs in infants and

children under five years of age but is rarely found in adults. 10 Age of children is a

predisposing factor for SSSS because of low immunity and immature renal ability to

clear toxins (exfoliative S. aureus toxin) . 1

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It is important to carry out further investigations to help make the diagnosis. A

simple examination that can be done is in the form of Gram staining. Gram staining

taken from the initial lesion, will show a positive cluster of Gram cocci. 1,13 Examination of

intact bullae culture on SSSS is usually sterile (no staphylococcus found), this is

consistent with the pathogenesis of hematogenous spread of toxins from distant foci of

infection. In the histopathological picture, there is separation of the epidermis between

the stratum granulosum and the stratum spinosum .1,14 In this case, the diagnosis was

made from clinical findings. No investigations were carried out because the patient was

not cooperative so it was not possible to do a biopsy.

Differential Diagnosis

The differential diagnosis of SSSS is impetigo bullosa and toxic epidermal

necrolysis (TEN). Staphylococcal scalded skin syndrome and bullous impetigo are

blistering skin diseases caused by ET, however in bullous impetigo, ET is only present

in the area of infection so that a bacterial culture can be obtained from the contents of

the blister. In SSSS, ET is spread hematogeneously and will potentially cause

epidermal damage to the farthest parts of the site. 1,15,16 Staphylococcal scalded skin

syndrome is differentiated from toxic epidermal necrolysis (TEN) based on the part that

is damaged, where SSSS occurs intraepidermally while TEN causes necrosis in the

entire epidermal layer (at the basement membrane boundary). Staphylococcal scalded

skin syndrome is of lower severity and does not involve mucous membrane erosion

when compared to NET. The predisposing factor for TEN is the use of drugs. 1,6,14

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Treatment

Management of SSSS is with antibiotics, skin care, and other supportive care to

maintain fluid and electrolyte balance. Hospitalization is required for SSSS treatment

because administration of intravenous antibiotics is the absolute therapy in the

management of SSSS.6 Staphylococcus aureus strains as the cause of SSSS are

Methicillin-sensitive, Penicillinase-resistant, so Beta-Lactam agents such as Cloxacillin,

Dicloxacillin, Oxacillin, Flucloxacillin, and Nafcillin are the antibiotics of choice.15,17 The

topical antibiotic options that are commonly used are Fusidic Acid or Mupirocin. 6

Corticosteroids are contraindicated because they can exacerbate SSSS. 4

Extensive impairment of skin protective function in SSSS lesions, causing fluid and

electrolyte disturbances. Proper fluid monitoring as well as skin care, is very useful to

accelerate healing. Compress the area of the lesion to clean the epidermal tissue that

has necrosis.1

In this case, the antibiotic of the Penicillin class, namely intravenous Ampicillin,

was given, but because intravenous access on day 3 of treatment was difficult to obtain,

it was replaced by the oral antibiotic Amoxicillin. Topical antibiotics in the form of

Mupirocin cream for 6 days then replaced with Fusidic Acid. Before administering

topical cream, the lesions were compressed with 0.9% NaCl solution every morning and

evening for 2 hours.

Prognosis

The prognosis for SSSS in neonates and children is generally good in the absence of

serious complications. With appropriate antibiotic management and adequate

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supportive therapy, the mortality does not reach 5%. The most common complications

in SSS are dehydration, sepsis, scarring, pneumonia, and post-streptococcal

glomerulonephritis.

Summary

The SSSS case report that was reported was a 5 months old baby girl with a

diagnosis based on history and physical examination, the supporting examination in the

form of a tissue biopsy was not performed due to the patient's uncooperative condition.

Management of these patients includes intravenous and oral antibiotics and supportive

skin care. The patient was discharged on the 7th day of treatment with a good general

condition and a noticeable improvement in the skin condition. The prognosis of this

patient is good because of proper management and parental compliance in maintaining

patient hygiene so that complications do not occur.

22
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