Staphylococcal scalded skin syndrome
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This case report describes a male infant who developed staphylococcal scalded skin syndrome (SSSS) caused by methicillin-sensitive Staphylococcus aureus producing exfoliative toxin B. He presented with fever, irritability, and an erythematous rash on his trunk and face that progressed to tender, painful skin and flaccid bullae. Standard treatment of systemic antibiotics was supplemented with application of Suprathel, a synthetic wound dressing, as a whole-body covering. This innovative treatment relieved pain, prevented heat loss and infection, accelerated healing, and did not require frequent changes.
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Staphylococcal scalded skin syndrome
- 2. Etiology • caused predominantly by phage group 2 staphylococci, particularly strains 71 and 55 • found in nasopharynx and, less commonly, the umbilicus, urinary tract, a superficial abrasion, conjunctivae, and blood • spreads hematogenously Nelson’s Textbook of Pediatrics. 19th Edition
- 3. Epidemiology • predominantly in infants and children younger than 5 years of age and rarely occurs in adults • Due to circulating antibodies and renal excretion of toxins • most cases are caused by type 71 strain (75%) • no differences in incidence based on gender nor economic status Nelson’s Textbook of Pediatrics. 19th Edition Schwartz, M. William. The 5 minute pediatric consult. 2nd ed.
- 4. PATHOPHYSIOLOGY • Caused by an exfoliative toxin: ETA and ETB • The toxins likely act as proteases that target the protein desmoglein-1 (DG-1) • Exotoxin causes separation of the epidermis beneath the granular cell layer.
- 8. DIAGNOSIS • Gram stain or Culture: from the remote site of infection • Skin biopsy or Frozen Section • PCR
- 9. Management
- 10. Pharmacologic • Systemic therapy, either orally, in cases of localized involvement, or parenterally, with a semisynthetic penicillinase-resistant penicillin, should be prescribed because the staphylococci are usually penicillin resistant • Clindamycin may be added to inhibit bacterial protein (toxin) synthesis
- 11. Review of Medication: • Hydroxyzine 2mg/ml, 2.5 ml every 6 hours PRN for pruritus • Mupirocin ointment, apply over nasal mucosa using cotton buds, 3x a day for 7 days • Erythromycin eye oitment, 1 strip to both lower lids 2x a day • Cloxacillin 250mg/ml, 2ml every 6 hours on an empty stomach, 1 hour prior to meals
- 12. Non-pharmacologic • The skin should be gently moistened and cleansed. • Application of an emollient provides lubrication and decreases discomfort. • Topical antibiotics are unnecessary.
- 13. Prognosis • Recovery is usually rapid, but complications such as excessive fluid loss, electrolyte imbalance, faulty temperature regulation, pneumonia, septicemia, and cellulitis may cause increased morbidity.
- 14. Blepharitis • Chronic inflammation of the eyelid • Associated with tear film disruption • Anterior – Affecting the anterior lid margin and eyelashes • Posterior – Affecting the Meibomian glands
- 15. Symptoms • Watery eyes • Red eyes • Burning sensation in eyes • Eyelids that appear greasy • Itchy eyelids • Red, swollen eyelids • Flaking of the skin around the eyes • Crusted eyelashes upon awakening • Eyelid sticking • More frequent blinking • Sensitivity to light • Eyelashes that grow abnormally (misdirected eyelashes) • Loss of eyelashes
- 16. Anterior Blepharitis • Staphylococcal bacteria • Seborrheic dermatitis • Manifestations – Foreign body sensation, burning sensation, matting of eyelashes, ring like formation around the lash shaft – Presence of madarosis, chalazion or hordeolum
- 17. Posterior Blepharitis • Inflammation of eyelids secondary to dysfunction of meibomian glands • Associations – Rosacea • Facial redness – Demodex mites • Affinity for hair follicles
- 18. Treatment • Proper hygiene – This condition is primarily treated with advocating cleaning of the affected area regularly – Warm water and mild shampoo for eyelashes • Antibiotics • Steroid Eyedrops • Artificial Tears
- 19. An innovative local treatment for staphylococcal scalded skin syndrome E. Mueller & M. Haim & T. Petnehazy & B. Acham-Roschitz & M. Trop
- 20. Case Report • Male infant – Different congenital malformations – Delivered via caesarean section at 36 weeks AOG due to oligohydramnios – Left lower limb deformity consisting of tibial and distal femoral aplasia, club foot and mirror foot – Multiple vertebral anomalies at different levels of the spine – Renal agenesis on the right and hydronephrosis on left kidney
- 21. Course in the Ward • 11 months of age – Severe diaper dermatitis with ulceration caused by intractable diarrhea secondary to short bowel syndrome and renal insufficiency – Microbial analysis: presence of Escherichia coli, Enterobacter cloacae, Klebsiella pneumoniae and Enterococcus faecalis, and S. aureus.
- 22. Course in the Ward • 14 months of age: – Developed fever, malaise and more irritable – Erythematous rash on skin of the trunk and facial area tender and painful skin and small flaccid bullae erupted – Cefuroxime IV, increased parenteral fluid support and transferred to children’s burn unit
- 26. Course in the Ward • Skin biopsy (right flank): mid-epidermal cleavage with minimal inflammation • Culture: methicillin-sensitive S. aureus (MSSA) producing ETB • Cefuroxime IV was adapted based on the impaired renal function • Suprathel® treatment as a whole-body dressing
- 28. Staphylococcal Scalded Skin Syndrome • Standard treatment: systemic antibiotics • Silver sulfadiazine is not recommended for SSSS • Steroids are contraindicated on the basis of both experimental and clinical evidence • Severe blistering skin diseases are better managed in burns units • Core temperature and room temperature need to be monitored carefully
- 29. Suprathel® • Synthetic copolymer consisting mainly of DL- lactide (>70%), trimethylene carbonate and ε- caprolactone • Imitates the properties of natural epithelium and consists of a membrane with 80% porosity • Permeable to oxygen and moisture
- 30. With Suprathel® in place: • Relieves pain • Prevents heat loss and secondary infection • Accelerates wound healing • Does not need to be changed (daily care is easier)
Notas do Editor
- Staphylococcal scalded skin syndrome is caused predominantly by phage group 2 staphylococci, particularly strains 71 and 55, which are present at localized sites of infection. Foci of infection include the nasopharynx and, less commonly, the umbilicus, urinary tract, a superficial abrasion, conjunctivae, and blood. The clinical manifestations of staphylococcal scalded skin syndrome are mediated by hematogenous spread, in the absence of specific antitoxin antibody of staphylococcal epidermolytic or exfoliative toxins A or B.
- Rarely in adults – because of increase circulating antibodies and better renal excretion of toxins
- Two exfoliative toxins (ETA and ETB) have been isolated ->-these toxins act at a remote site leading to a red rash and separation of the epidermis beneath the granular cell layer. The toxins likely act as proteases that target the protein desmoglein-1 (DG-1), an important cell-to-cell attachment protein found only in the superficial epidermis.The epidermolytic toxins appear to produce the granular layer split by binding to desmoglein I within desmosomes. Evidence suggests that the toxins are members of the trypsin-like serine protease family and may exert their action through proteolysis. The relative quantity of DG-1 in the skin differs with age and may partially explain the increased frequency of staphylococcal scalded skin syndrome in children younger than 5 years.
- It begins with the S. aureus bacteria entering a wound and entering the circulatory sytem. Then passing systemically, into the dermal layers and a step procress of the effect that S.aureus toxins create. The baby in the corner represents on a large scale the physical apparance of Scalded Skin Syndrome.
- The superficial epidermis is composed of the strateumcornuem (uppermost layer), and an underlying layer which contains granular, spinous and basal cells. The basal lamina separates the epidermis from the dermis. The top layer of epithelial cells express Dsg1, while deeper endothelial cells express both Dsg1 and Dsg3 in their desmosomes. Desmosomes connect the cells of the epidermis. In cases of SSSS the staphylococcal exfoliative toxin A (ETA) cleaves Dsg1, disrupting the desmosomes and causing the superficial epidermis to 'split away'.
- Differential distribution of desmoglein isoforms in the epidermis [80] explainsthe exfoliative-toxin-induced splitting at the stratum granulosum. Schematic representationof the desmoglein distribution in (A) healthy skin and (B) skin exposed to exfoliativetoxin. In all strata, except the stratum granulosum, the exfoliative-toxin-mediatedhydrolysis of desmoglein 1 (Dsg-1) is compensated by desmoglein 3 (Dsg-3). Dsg-3 isabsent in the stratum granulosum, which explains the cell detachment and the splitting ofthe epidermal layers upon the hydrolysis of Dsg-1.
- A Gram stain and/or culture from the remote infection site may confirm staphylococcal infection.Intact bullae are consistently sterile, unlike those of bullous impetigo, but cultures should be obtained from all suspected sites of localized infection and from the blood to identify the source for elaboration of the epidermolytic toxins. The subcorneal, granular layer split can be identified on skin biopsy. Absence of an inflammatory infiltrate is characteristic. A biopsy of the affected area will demonstrate separation of the epidermis at the granular layer.In cases that demand a rapid diagnosis, the exfoliated corneal layer can be seen on a frozen biopsy specimen of the desquamating epidermis. Frozen section of the peeled skin confirms the site of cleavage as superficial. Toxic epidermal necrolysis (TEN) shows deeper cleavage below the epidermis.A polymerase chain reaction (PCR) serum test for the toxin is available.Typing of staphylococcal isolates for phage and subtype and the presence of exotoxin production is usually not necessary but is available at some centersWhite blood count (WBC) may be elevated; however, often WBC is normal.Erythrocyte sedimentation rate (ESR) frequently is elevated.Electrolytes and renal function should be followed closely in severe cases where fluid losses and dehydration via denuded skin are a concern.
- main problem regarding nursing care was severe diaper dermatitis with ulceration caused by intractable diarrhoea (at least ten stools a day) as a result of the combination of the short bowel syndrome and the renal insufficiency. The diaper dermatitis proved to be intractable to any local treatment.
- Under anaesthesia large sheets of epidermal detachment were removed and swabs taken (which remained sterile) (Fig. 1). The total body surface area affected was more than 50%. Mucous membranes and nails were not affected. Nikolsky’s sign was positive.
- On the cleaned, oozing superficial red wounds, Suprathel® was applied above itand one layer of paraffin and absorbent gauze, secured with an elastic netting
- The next day, the wound check in the OR revealed new lesions all over the body, so that, in the end, 90% total body surface area was affected after 36 hours. Suprathel® wound dressing was extended to the whole body with the exclusion of the genital region due to the frequent loose stools.
- The intravenous dosage of the antibiotic was adapted based on the impaired renal function (serum creatinine between 266 and 354 μmol/L, normal range 26–44 μmol/L).
- With Suprathel® treatment as a whole-body dressing, the blister formation subsided and complete resolution occurred within five days, without scarring. The patient was transferred back to the Department of Neona- tology after seven days at the paediatric burns unit. The intravenous antibiotic therapy was continued for a total course of 14 days.
- In severe cases, intravenous therapy with anti-staphylococcal antibiotics is required Silver sulfadiazine is not recommended for SSSS because of enhanced systemic absorption through denuded skinCore temperature and room temperature need to be monitored carefully, as thermal dysregulation is com- mon; even though the patient is febrile, peripheral vasodi- latation adds to loss of heat and may cause a drop in the core temperature.
- In our case, the main focus of attention at the burns unit was given to the denuded skin and the subsequent implications of such a large wound area.
- In our case, the main focus of attention at the burns unit was given to the denuded skin and the subsequent implications of such a large wound area