Scalded Skin Syndrome Case Study
Staphylococcal Scalded Skin Syndrome in a Newborn
D Jeyakumari,R Gopal,M Eswaran,1 and C MaheshKumar1
Department of Microbiology and Paediatrics, Madagadipet, Pondicherry, India, Affiliated to Pondicherry University
1Department of Sri Manakula Vinayagar Medical College and Hospital, Madagadipet, Pondicherry, India, Affiliated to Pondicherry University
Address for correspondence: Dr. Jeyakumari D, E-mail: ni.oc.oohay@eroladducsbaliarak
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A six-day-old newborn was admitted with exfoliating erythematous lesions over the face, of two days duration. The lesions spread to the rest of the body during the next two days. A diagnosis of Staphylococcal Scalded Skin Syndrome (SSSS) was made clinically and confirmed by isolation of Staphylococcus aureus from a blood sample. The child responded to Injection vancomycin and no fresh lesions were seen after the next 48 hours. However the child developed severe pneumonia and left against medical advice.
Keywords: Staphylococcus aureus, Scalded skin syndrome, Exfoliative toxins
Staphylococcal Scalded Skin Syndrome (SSSS) is an extensive exfoliative dermatitis that occurs primarily in newborns and previously healthy children. This syndrome is caused by Staphylococcal exfoliative or epidermolytic toxin produced by Staphylococcus aureus. The severity of the disease varies from being a localized skin lesion to a more extensive generalized condition, characterized by cutaneous erythema followed by profuse peeling of the epidermal layer of the skin. The duration of the disease is brief with a low mortality rate among children with prompt treatment. A case of SSSS in a newborn is reported because of its rare occurrence.
A six-day-old newborn was admitted with erythematous lesions over the face, of two days duration. The child was a full-term, normal baby. The child developed an erythematous rash on the fourth day of birth. The lesion started behind the ear and spread to other parts of the face and neck within the next two days. There was no history of administration of any drugs after birth. On examination at admission the child was toxic, febrile with erythematous lesions around the mouth, nose, and over the neck [Figure 1]. The oral mucosa was normal. The lesions spread to the hands, gluteal region, thighs, and legs over the next two days [Figures 2–4]. A culture of the blood sample collected on the day of admission yielded a pure growth of Staphylococcus aureus, which was sensitive to oxacillin, cefazolin, cefatoxime, vancomycin, netilmycin, ticarcillin, and ciprofloxacin. Swabs collected from the cutaneous lesions on the day of admission did not yield any growth. In view of the extension of the lesions to other areas of the body the child was put on injection vancomycin and topical soframycin in addition to cefatoxime started earlier. A clinical diagnosis of Staphlococcal Scalded Skin Syndrome (SSSS) was made. The lesions healed over the next two days and no new lesions were observed. The child became afebrile and was feeding well. However the child developed tachypnea with a respiratory rate of 76/minute on the sixth day of admission and had an episode of generalized seizures, which responded to intravenous calcium gluconate. An X-ray of the chest was suggestive of right upper lobe pneumonitis. An ECG showed sinus tachycardia with right ventricular hypertrophy. Repeat blood culture on day six of admission was sterile. The patient developed a swelling over the right ankle on day seven [Figure 5]. Aspirated pus yielded coagulase-negative Staphylococcus. A nasal swab of the mother yielded a pure growth of Staphylococcus aureus.
Lesions over the back of the hand
Extensive exfoliations over the gluteal region
Secondary infections over the right foot
Staphylococcal Scalded Skin Syndrome describes a spectrum of superficial blistering skin disorder caused by the exfoliative toxins of Staphylococcus aureus. In a severe form, the exfoliation can spread to cover the entire body surface.  SSSS particularly affects infants and young children. Lack of protective antibodies to exfoliative toxins A and B (ETA and ETB) and immature renal function, which impairs the ability to excrete the toxin have been suggested as reasons. In infants and young children, potentially fatal complications include hypothermia, dehydration, and secondary infections. Three forms of the Staphylococcal skin disease have been described in neonates, namely, SSSS, Bullous Impetigo (BI), and Staphylococcal Scarlet fever. SSSS and BI have many features in common. However, compared with BI the skin lesions of SSSS are larger, Staphylococcus aureus is less frequently isolated, and less inflammatory infiltrate in the skin lesions is noticed. Characteristically SSSS consists of diffuse erosions with epidermal separation in the submucosal layer through the granular layer. The differential diagnosis of SSSS includes drug-induced toxic epidermal necrolysis, epidermolysis bullosa, bullous mastocytosis, herpetic lesions, and neonatal pemphigus. Clinically the SSSS mimics toxic epidermal necrolysis (Lyell's syndrome). The typical features of SSSS are involvement of periorificial face, de-epithelialization of friction zones, and absence of mucosal involvement. However, in toxic epidermal necrolysis (Lyell's syndrome) there is a severe involvement of visible mucosa and also the respiratory, gastrointestinal, and urinary tract mucosae.
In the case reported, the characteristic skin lesions and failure to isolate Staphylococcus aureus from the lesions, but from blood culture, absence of any mucosal lesions, and no history of administration of drugs was suggestive of SSSS. With prompt treatment the progression of the lesions was arrested and no new lesions occurred. In the case described the child responded to injection vancomycin with no new lesions and became apyrexic within 48 hours. However, the child subsequently developed arthritis and pneumonia probably due to secondary infection. The child was discharged on request by the parents and was lost to follow up.
Staphylococcal Scalded Skin Syndrome can occasionally lead to serious complications like pneumonia, septic arthritis, hypothermia, dehydration, and secondary infections. With appropriate management, however, mortality due to SSSS in children remains below 5% in comparison to about 60% in adults. Therefore, early diagnosis and appropriate treatment can prevent the mortality associated with these complications.
We wish to thank our Director Dr. D. Rajagovindan for giving permission to send this case report for publication.
Source of Support: Nil
Conflict of Interest: None declared.
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Key Clinical Message
Benign impetigo can progress into a potential fatal staphylococcal scalded skin syndrome (SSSS) if prompt diagnosis and correct therapy is not established rapidly. Local and systematic antibiotics as well as Lactulose are crucial in order to stop SSSS from progressing. Burns units should be involved when skin lesions are extensive.
Keywords: Intensive care, SSSS, staphylococcal scalded skin syndrome
Staphylococcal Scalded Skin Syndrome (SSSS) is a vesiculating skin disease often originating from bullous impetigo. SSSS is normally found in children under the age of 5 and is usually caused by the toxins type A or B coming from Staphylococcus aureus1. The toxin spreads hematogenously and involves the superficial epidermis. It heals within 10–14 days without scarring 1. Mortality in infancy is low (4%) 1. Burn units should be involved when skin lesions are extensive 1.
We present a case of a 5‐year‐old girl with rapidly progressing SSSS covering most of her body's surface area.
Day 1: A 5‐year‐old healthy girl presented with a sore bullae in the nasolabial line on the right side which measured 1 × 1 cm. The bullae erupted and left a red denuded area. She also had reduced appetite, seemed irritable, and complained of having a sore throat. When drying the nose, the outermost layer of the skin sloughed off (positive Nikolsky's sign. Fig. 1B). Signs of early bilateral conjunctivitis were also present. At this stage, she did not have a fever. The mother consulted the emergency department. With the suspicion of impetigo, the doctor prescribed Fucidin ointment.
Signs of early conjunctivitis (A) and desquamation of skin (Nikolsky's sign) in the right nasolabial line (B) Desquamation of skin from cubital fossa's on second night.
Day 2: With the symptoms not resolving, the mother took the child to her local GP. On continued suspicion of impetigo, the GP did not change the treatment with Fucidin ointment. Within hours, the bullae spread to the right clavicle and on the backside of the right ear. The patient was still afebrile. With the symptoms advancing, the mother took the child to the emergency department where the child was prescribed a course of Flucloxacillin and sent home. Blood samples clarifying infection counts and fluid electrolytes were not measured.
Same night, the patient awoke with fever, pains, and desquamation of the skin around eyes and mouth, on the back and gluteal area, both axils, cubital fossa (Fig. 1C and D) and popliteal. The rash covered about 10% of the body's surface area. The child was admitted to the local hospital and treated with intravenous Flucloxacillin. Prior to treatment with Flucloxacillin, samples from skin lesions, nasal cavity, perineum, axils, and trachea were obtained and sent to culture growth and microscopy. S. aureus was identified a week later from nasal cavity and pharynx, while samples taken from skin lesions, axils, and perineum were without the presence of S. aureus.
Day 3: After conference with the specialized unit for burn injuries, a treatment of oral Fucidin, intravenous Penicillin, Lactulose, and fluid maintenance was commenced. The patient was then transferred by helicopter to the Clinic of Burn Injury. At arrival, the bullae formation now covered 55% of the body's surface area, including the eye and mouth area, ears, neck, back, gluteal, axilla, cubital fossa's, wrists, hands, genitalia, the groin, popliteal, and ankles (see Fig. 2). The patient seemed fatigued, febrile, and in intense pain. She was placed in a sterile, heated burns injury ward. A central vein catheter was inserted in general anesthesia since a usual peripheral vein catheter could not be inserted. Infection counts and fluid electrolytes were measured in blood samples and results unremarkable. Skin biopsy was performed and SSSS confirmed the following day. Feces and blood were sent to culture growth and microscopy. The presence of S. aureus was confirmed within 2–3 days (gram stain was used after identification of culture growth from blood and prior to microscopy). The patient was given sufficient pain relief. The conjunctivitis was treated with Chloramphenicol eye drops. Fluid maintenance aiming at an hourly diuresis of 1 mL/kg per hour was commenced and the treatment with intravenous Penicillin, oral Fucidin, and Lactulose continued.
Pictures illustrates the progression of SSSS on the third day. Desquamation of skin covering 55% of the body's surface area.
The patient was in recovery the following day and stayed hospitalized until the wounds heeled with incrustation. On the 10th day she was discharged. At 1‐month follow‐up examination, no scarring was obtained.
Cells in epidermis are held together by the protein desmoglein 1 1. In SSSS, the toxins from S. aureus chop the superficial fragments of desmoglein 1, which results in loosening of epidermis and the distinctive bullae formation 1. The toxins produced by S. aureus can be detected by polymerase chain reaction (PCR) within 3 h ensuring the diagnosis 2. Skin biopsy is also diagnostic revealing an intraepidermal cleavage, with splitting of stratum granulosum, which may contain acantholytic cells. The epidermis appears unremarkable and dermis without inflammatory cells 1, 3. S. aureus toxin‐mediated infection are evident in children. As presented in current case report, S. aureus does not occur in the skin lesions, but are confined to the site of infection, primarily the nasopharynx. Unlike present case, blood cultures do not typically help in diagnosis of a child with SSSS, as they are usually negative. Contrary, S. aureus in an adult's blood cultures are commonly positive 1. The patient's condition can be complicated by a secondary infection (pneumonia, sepsis, and cellulitis), dehydration, and consequential electrolyte imbalances 1, 2. With positive Nikolsky's sign and scalded skin appearance, SSSS may resemble other blistering disorders as toxic epidermal necrolysis (TEN) and Steven–Johnson Syndrome (SJS). However, SSSS can be differentiated from TEN by the lack of mucous membrane involvement as well as by more superficial epidermal peeling, which is in contrast to the full thickness denudation found in TEN and SJS. SSSS is most common in children and not a result of medication as in TEN/SJS. Although similar histopathologically to TEN, SJS needs at least two mucous membrane involvements, and unlike TEN, the skin involvement is generally limited to less than 30% body surface area. Skin biopsy can rapidly differentiate between SSSS and TEN/SJS 4.
Early and correct treatment that includes both local and systemic antibiotics as well as Lactulose is crucial in order to stop SSSS from progressing. In children, the toxins from S. aureus cannot be excreted with the urine, as the kidneys are immature. Even though the patient is not constipated and Lactulose is not a part of the standard treatment against SSSS in most hospitals around the world, it is our opinion that Lactulose and the subsequent acceleration of gastrointestinal transit enable the patient to excrete S. aureus via the colorectal tract faster and easier. One study reported increased titers of S. aureus antibody (indicating increased bacterial load) in serum in 57 functional constipated patients, which was effectively treated with laxatives 5. However, it is not without risk to use laxatives in a nonconstipated patient with the need of well hydration. Thus, healthy persons must know the importance of proper timing, hydration and dosing, and how to administer laxative safely. The present case report motivates further research on the hypothesis that laxatives induce gastrointestinal elimination of S. aureus and its toxins.
The learning from this case report is the imperativeness to have an early diagnosis of SSSS, correct treatment with antibiotics, and fluid maintenance to ensure a prompt and complete recovery. Patients suspected of suffering from this condition should see a medical doctor immediately and burns units should be involved when skin lesions are extensive 1.
Written informed consent was obtained from the parents for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.
Conflict of Interest
Clinical Case Reports 2016; 4(4): 416–419
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