Main Text:
Background:Staphylococcal Scalded Skin
Syndrome (SSSS) is an exfoliative skin disease that usually occurs due
to a toxin produced by a Staphylococcal aureus infection [1].
Toxin a or b from S. aureus spread hematogenously and have been
shown to lyse the cell-cell adhesion molecule desmoglein-1 in the
superficial epidermis, causing intraepidermal splitting and thus a loss
of structural integrity [2]. SSSS primarily occurs in children and
neonates and is rare in the adult population [3, 4, 5] We present a
case of a 20 month old patient who presented with progressive SSSS and
recurrence of SSSS to emphasize the role of early treatment and
diagnosis in reducing morbidity of the disease.
Case Presentation
Initial Admission:
Day 1: A 19 month old African American male presented to the ED with
erythematous patches and blistering on face and neck as well as
erythematous patches on buttocks (Figure 1). Patient had a history of
atopic dermatitis mostly affecting bilateral antecubital fossa. The
patient had no new medications prior to this event and was up to date on
relevant immunizations.
Per caregivers who were present at ED, the patient was also irritable,
had reduced appetite, and appeared fatigued 3-5 days before
presentation. The patient also experienced two days of afebrile dry
cough, rhinorrhea, and sinus congestion, increased scratching of the
antecubital fossa due to his atopic dermatitis. Upon rubbing of the
perinasal and perioral area in the ED, the uppermost layer of skin
sloughed off with minimal pressure (positive Nikolsky’s sign). On
physical exam, erythema and edema were present. The mucous membranes of
the mouth and pharynx were not affected. The patient was apyretic. The
patient’s initial WBC was 8.3 x 10^9 /L. Further investigation with
blood and ear wound cultures were obtained. A diagnosis of SSSS was made
based on history and clinical features. The diagnoses of bullous
impetigo and TEN were also considered, but the lack of mucous membrane
involvement in our patient made SSSS more likely. A skin biopsy was not
performed as the clinical presentation was consistent with SSSS.
The patient received IV nafcillin 40mg/mL every 6 hours after admission
as well as intravenous fluids. For pain control, the patient was given
ibuprofen and Tylenol as needed. The patient’s wounds were covered with
sterile bandages.
Days 2-3: After discussion with the caregivers, on day 2 the patient was
started on IV clindamycin 30 mg/kg/day to target the exfoliative toxin
of S. aureus. After 48 hours, the results of the blood and ear
wound cultures were negative and the urine analysis was clear. The
patient’s pain was controlled. However, the patient’s caregivers noticed
increased periorbital edema without drainage on day 2. This was thought
to be due to third spacing in the setting of inflammation and fluids
were decreased to KVO.
Of note, the patient’s WBC count of 5.0 X 10^9 /L (ANC 0.9) on
hospital day 3 was thought to be caused by IV nafcillin. Repeat CBC
within 24 hours showed WBC count 5.1 (ANC 1.2). On day 3, periorbital
edema was decreased and skin lesions were in the process of healing with
crusting (Figure 2). The patient was taking adequate oral intake, and
the IV antibiotics were transitioned to oral cephalexin and clindamycin
was discontinued. The patient was discharged on appropriate 7 day course
of oral cephalexin, topical mupirocin and close outpatient pediatric
follow-up.
Readmission, 3 weeks later: Subsequently, the now 20 month old patient
presented to the ED with erythematous patches on face, neck,
hyperpigmentation on buttocks consistent with SSSS. Patient’s caregivers
stated that patient’s skin was clear for 2 weeks after initial admission
and treatment, and the patient continued to scratch antecubital fossa at
night due to atopic dermatitis. The patient’s mother noticed an
erythematous patch behind the ear which had worsened for the past two
days, so she brought the patient to the ED. At readmission, there was
also no involvement of mucosal membranes and thus no concern for SJS.
CBC and CMP were obtained in the ED and were in the normal range except
for a low Na, 133 mmol/L. Blood cultures were obtained showing no growth
after 48 hours; wound cultures identified no organisms. Initially the
patient was given IV nafcillin for MSSA coverage because during the
prior admission this treatment healed the rash.
After discussion with the Pediatric Infectious Disease team, on day 2
the patient was switched from IV nafcillin to a ten day course of PO
Linezolid. The Pediatrics ID team was concerned that, due to the rapid
recurrence of SSSS, this patient may be experiencing a rare case of MRSA
caused SSSS. Thus, the coverage needed to be broadened.
On day 3, the patient had fewer new lesions and previous lesions
appeared to be healing with scabbing and hyperpigmentation. He was
afebrile during his entire hospital stay. The patient was also well
appearing with adequate PO intake during the hospital stay and was
discharged on day 3 with close outpatient pediatric follow up. The
patient’s mother was appropriately concerned for immune deficiency given
the recurrence of SSSS. Pediatrics Infectious Disease commented that his
readmission was more likely due to MRSA rather than MSSA and that is the
reason that SSSS recurred. The patient was also otherwise a normally
developing child with excellent growth. The patient’s mother was aware
that she needed to return should symptoms worsen or reoccur.
Discussion:
In this case report, the prompt diagnosis and treatment of SSSS during
the first ED admission allowed the patient to heal, and the subsequent
readmission with broadened antibiotic coverage allowed for timely
discharge and effective treatment.
SSSS, or Ritter’s disease, is a rare disease process that typically
occurs in children. The preceding S. aureus infection spreads in
the bloodstream and lyses the desmoglein-1 proteins in the stratum
granulosum layer of the epidermis [1, 2, 6]. This separation of the
anchoring desmosomes leads to the exfoliative presentation of SSSS. SSSS
typically arises from an area of infection, such as impetigo, bacterial
conjunctivitis, or iatrogenic wounds [7]. The susceptibility of
children to acquisition of SSSS has been postulated to be a result of
the lack of protective antibodies S. aureus toxins and/or the
insufficient excretion of exotoxins from children’s kidneys [1].
The diagnosis of SSSS is mainly clinical. Prodromal symptoms of
irritability, skin pain, fever, and poor feeding are common. Cutaneous
erythema may initially present in the skin folds of the neck, axillae,
gluteal folds before becoming generalized within the first 48 hours of
presentation [7]. On exam, the skin may have flaccid bullae that
erode upon minimal pressure. Additionally, superficial desquamation
occurs due to the exotoxin’s lytic mechanism [6]. There is typically
quick progression of the disease process.
The standard treatment of SSSS includes IV antibiotic against
staphlycoccal species (eg. Nafcillin, oxacillin) and supportive care
[6, 7, 8, 9]. Empiric treatment with penicillinase-resistant
penicillins is done initially, with broadening for MRSA coverage
considered when clinical improvement is not observed or in communities
with high MRSA incidence [10]. In our case, MRSA coverage was added
(PO linezolid) after initial clearing and improvement was followed by a
recurrence of SSSS. Clarithromycin can be given to patients with a
penicillin allergy [8]. Other therapies such as IV immunoglobulin
have been suggested to antagonize the exfoliative toxins of SSSS
[11]. Patients may continue to have skin pain following treatment as
well as post-inflammatory hypopigmentation or hyperpigmentation during
the healing process [6]. SSSS carries a risk of progression to
sepsis if not recognized and treated early. Other complications include
secondary infection, dehydration, electrolyte imbalance, and death [5,
12]. Recurrence is rare but can occur in young infants and neonates
[13].
The differential diagnosis for SSSS includes TEN (Toxic Epidermal
Necrolysis) and SJS (Steven’s Johnson Syndrome) [5]. Both TEN and
SJS have mucous membrane as well cutaneous involvement, which was not
present here. Bullous impetigo is also on the differential diagnosis,
but bullous impetigo has localized skin infection rather than
hematogenous spread. The former has more limited involvement and a less
severe clinical presentation [6].
Conclusion:
This case report highlights the importance of prompt evaluation and
antibiotic treatment as well as the consideration of MRSA in SSSS. More
research is necessary to elucidate the prevalence and resistant
staphylococcal strains and therapies for SSSS caused by MRSA. This case
report aims to further support research in exfoliative skin conditions.