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CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 192-196

Staphylococcal-Scalded skin syndrome: A case series


Department of Skin & VD, MGM Medical College, Aurangabad, Maharashtra, India

Date of Submission15-Jan-2020
Date of Decision13-Jul-2020
Date of Acceptance12-Oct-2020
Date of Web Publication26-Aug-2021

Correspondence Address:
Arpita Arghya
D019, Platinum Lifestyle Apartments, JP Nagar 8th Phase, Bangalore, Karnataka - 560 083
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_4_20

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  Abstract 


Staphylococcal-scalded skin syndrome (SSSS) is also known as Ritter's disease. The symptoms produced by the strains of staphylococcus occur due to exfoliative toxins. Here, we have presented a case series of four children aged <2 years, who were admitted in MGM Medical College and Hospital, Aurangabad, for fever in association with superficial fluid-filled lesions leaving large denuded areas behind Nikolsky's sign – positive. The mucosal area was spared. Blood culture served as diagnostic test, besides clinical history and examination. All the children were treated with cephalosporin and responded well. Hydration was maintained with topical mupirocin over the eroded areas. A high degree of suspicion has to be there for SSSS. We emphasize the importance of interdisciplinary management, association with dermatologist, pediatrics, and plastic surgeon for better outcome. Although considered rare, we came across four cases of SSSS with classical presentation.

Keywords: Nikolsky's sign, staphylococcal-scalded skin syndrome, Staphylococcus aureus


How to cite this article:
Gulanikar A, Arghya A. Staphylococcal-Scalded skin syndrome: A case series. Clin Dermatol Rev 2021;5:192-6

How to cite this URL:
Gulanikar A, Arghya A. Staphylococcal-Scalded skin syndrome: A case series. Clin Dermatol Rev [serial online] 2021 [cited 2021 Dec 1];5:192-6. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/2/192/324561




  Introduction Top


Staphylococcal-scalded skin syndrome (SSSS), also known as Ritter's disease, neonatal pemphigus, or acute staphylococcal epidermolysis, is a life-threatening rare disease. It is an exfoliative skin disease caused by exotoxins produced by staphylococcus species. In this infection, the skin surface gets peeled off, resembling burned skin by hot liquid.[1] Primary infection predominantly begins during the first few days of life in the umbilical area and in children-face is a typical site. SSSS mainly occurs in the children of age 48 h to 2 years. The incidence of SSSS is low and is listed as one of the rare disease. Seen frequently in the age group <2 years, but is also encountered in adults with immunocompromised status and renal diseases.[2]


  Report of Cases Top


The data were collected from patients admitted in the tertiary care hospital. Prompt development of blisters and formation of raw areas, irritable child and positive Nikolsky's sign was the common features in all the cases. Laboratory investigations showed leukocytosis and positive blood culture for Staphylococcus aureus.

Case 1

A 1-year-old boy, born of normal vaginal delivery was admitted for fever of acute onset, moderate grade which was associated with appearance of generalized erythematous skin lesions present over the face, neck, trunk, and groin area. The lesions first appeared on the face which spread to involve neck, trunk, and groin area. There was the formation of large superficial blisters which ruptured on slightest pressure leading to large denuded and eroded skin [Figure 1] and [Figure 2]. There was no similar complaint in past/family. The patient was not on any medication before reporting to our outpatient department (OPD). On cutaneous examination, there were multiple vesicles and bullae that involved the chest, neck, back, and groin area. Few dried crusted lesions with single erosion was present over the chest. Nikolsky's sign was positive.
Figure 1: There are multiple vesicle and bullae that involved the chest. Few crusted lesions with erosion are present over the chest

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Figure 2: Multiple flaccid fluid-filled vesicle and bullae present over the back

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Case 2

A male child aged 2 years born of normal vaginal delivery, developed moderate grade fever associated with cold and cough. He then started developing large superficial blisters on the trunk, which spread to involve face, bilateral upper limbs, and bilateral lower limbs [Figure 3], [Figure 4], [Figure 5]. The lesions ruptured on slightest pressure leading to large denuded raw areas. No similar complaints in past/family. The patient was not on any medication before reporting to our OPD. Cutaneous examination showed multiple vesicles and bullae on the face, trunk, bilateral upper limb, and lower limbs which eventually healed to form scaling with underlying erythematous base over those lesions. Nikolsky's sign – positive. The patient was given appropriate treatment within 7 days of treatment [Figure 6] and [Figure 7].
Figure 3: There is diffuse erythema with scaling present over the nape of the neck, back, shoulders, and buttocks

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Figure 4: Multiple erosions are present over the face and trunk. New lesions showed typical scaling over erythematous base

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Figure 5: Few erythematous patches with surrounding peeling off skin present over the trunk

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Figure 6: The patient showed complete resolution of lesions within 7 days over the trunk

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Figure 7: Resolution of lesions were seen within 7 days, but there is the presence some scaling over the nasal bridge and periorbital area

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Case 3

A 6-month-old boy, born of normal vaginal delivery, had started developing vesicles over face, for 3 days. There was a history of fever associated with the appearance of vesicles which on slightest pressure lead to the formation of raw denuded areas [Figure 8]. H/o similar complaints in other siblings. The patient was not on any medication before presenting to our OPD. Cutaneous examination showed multiple eroded skin with thick adherent crusting over erythematous base present over the face. Scaling is present on the trunk. After 5 days, patient had responded well to the treatment [Figure 9].
Figure 8: Multiple eroded skin with thick adherent crusting over erythematous base present over the face

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Figure 9: Near complete resolution of scales was present over the face at the end of the 5th day. Few crusts were seen in the nasal alae and the vermillion border of the upper lip

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Case 4

A male infant born of normal vaginal delivery presented to us with the complaints of fluid-filled lesions in the last 48 h which started over the trunk and spread to involve the whole body, which lead to the formation of raw eroded areas. Association of fever was seen. Cutaneous examination showed multiple eroded skin. The tender skin has gathered into folds, and its shrinkage had left behind raw areas that were extremely painful. Nikolsky's sign was positive [Figure 10], [Figure 11], [Figure 12]. Posttreatment on the 10th day when the patient was brought for follow-up, patient had responded well to the treatment prescribed [Figure 13].
Figure 10: Multiple eroded skin lesions with underlying red raw areas, surrounding skin has gathered into folds, and its shrinkage had left behind raw areas

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Figure 11: Multiple erosion with underlying erythematous base with few crusting present over the nasal cavity, perioral area, and neck

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Figure 12: Tender skin that had gathered into folds and its shrinkage had left behind raw areas

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Figure 13: Complete resolution of skin lesions at the 10th day of visit

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Treatment

All patients were treated with injectable cephalosporin and supportive treatment. Topically, mupirocin 2% was given over the raw areas. Zinc solution and multivitamin drops were supplemented additionally. The response was seen within 3 days. Patients recovered within 7 days of initiation of treatment. All patients responded well [Figure 6], [Figure 7], [Figure 9] and [Figure 13].


  Discussion Top


SSSS, also known as Ritter's disease, is a superficial blistering disorder primarily affecting the infants and children because the toxin liberated by the bacteria is not neutralized by the immature kidney.[3] Bacterial colonization and carrier state play an important role in pathogenesis. The pathogenic agent is exfoliative toxin, produced by S. aureus which is also seen in in bullous impetigo.

The difference in both is the disease extent, action of toxin whether at local level or at systemic level and amount of toxin released. There are two types of exotoxins: type A and B. Exotoxin A is most producing one. Phage group II is the most common strain of S. aureus isolated from the patient of SSSS.[4],[5] The exfoliative toxins (ET) have exquisite specificity in causing loss of desmosome-mediated cell adhesion.[6] ET A and B are serine proteases that cleave desmoglein leading to characteristic skin lesions.[7] The split is seen along the granular layer leading to intra-epidermal split, like bullous impetigo, but in bullous impetigo pronounced inflammatory cells infiltrate consisting mostly of neutrophils.[4]

SSSS has the sudden onset of painful, tender, and erythematous skin accentuated in periorificial and flexural areas. 2–3 days after the onset of disease, there is the appearance of flaccid blisters progressing to erosions, leading to loss of epidermis over a large area. Here epidermis peel like a scald which can be extended.[8]

The characteristic appearance of rash with fever makes the diagnosis of clinical ground.[8] Clinical variants are of two types-generalized and localized SSSS. Localized SSSS seems to favor the flexures, in particular the axillae, groin, and limb flexures. Healing of which classically leaves wrinkled desquamating skin with hyperpigmentation. The confirmation is done by swab from skin lesions and orificial areas. Blood culture is confirmatory, as done in our case. The skin biopsy shows a split into the granular layer with inflammatory cells infiltration. The diagnosis is made in our case based on (a) clinical findings (b) blood culture. In our four cases, we however did not encounter methicillin-resistant S. aureus (MRSA). However, if MRSA is suspected, vancomycin or linezolid is indicated.[9] Clindamycin is recognized as being useful in staphylococcal infections.[9] Patient developing SSSS is treated in the same manner as burn patient, i.e., maintenance of hydration, topical wound care together with interdisciplinary team of pediatrician and dermatologists.

Hence, if there is the appearance of fever with exanthema, especially in children, the dermatologists should especially consider SSSS TEN and drug reaction as the differential diagnosis. Early prompt treatment gives good prognosis, but delay in treatment, improper diagnosis, and in immunocompromised, the disease may progress and lead to cellulitis, septicemia poststreptococcal glomerulonephritis, and other complications leading to death.[10]

This article has tried to shower the light that even though the disease is labeled as a rare disease, high index of suspicion, and early prompt treatment gives excellent outcome.

Declaration of patient consent

The author certifies that all appropriate consent forms were obtained from the patient's parents for the publication of the case report. Consent was also given for images and other clinical information to be reported in the journal. They were assured that the patients' names and initials will not be published and though every effort will be made to conceal the patient's identity, anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Itani O, Crump R, Mimouni F, Tunnessen WW. Picture of the month: SSSS. Am J Dis Child 1992;146:425-6.  Back to cited text no. 1
    
2.
Braunstein I, Wanat KA, Abuabara K, McGowan KL, Yan AC, Treat JR. Antibiotic sensitivity and resistance patterns in pediatric staphylococcal scalded skin syndrome. Pediatr Dermatol 2014;31:305-8.  Back to cited text no. 2
    
3.
Pathak S, Sethi A, Sharma N, Rathod GB, Rasania M, Gandhi DJ. Staphylococcal scalded skin syndrome in a newborn: A case report. IAIM 2015;2:66-9.  Back to cited text no. 3
    
4.
Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: Diagnosis and management. Am J Clin Dermatol 2003;4:165-75.  Back to cited text no. 4
    
5.
Rieger-Fackeldey E, Plano LR, Kramer A, Schulze A. Staphylococcal scalded skin syndrome related to an exfoliative toxin A and B-producing strain in preterm infants. Eur J Pediatr 2002;161:649-52.  Back to cited text no. 5
    
6.
Lillibridge CB, Melish ME, Glasgow LA. Site of action of exfoliative toxin in the staphylococcal scalded-skin syndrome. Pediatrics 1972;50:728-38.  Back to cited text no. 6
    
7.
Ladhani S. Understanding the mechanism of action of the exfoliative toxins of Staphylococcus aureus. FEMS Immunol Med Microbiol 2003;39:181-9.  Back to cited text no. 7
    
8.
Braunstein I, Wanat KA, Abuabara K, McGowan KL, Yan AC, Treat JR. Antibiotic sensitivity and resistance patterns in pediatric staphylococcal scalded skin syndrome. Pediatr Dermatol 2014;31:305-8.  Back to cited text no. 8
    
9.
Darnstadt GL, Behrman RE, Kliegman RM, Jenson HB. Cutaneous bacterial infections. In: Nelson Textbook of Pediatrics. Philadelphia, Pennsylvania, USA: W. B. Saunders Co.; 2000. p. 2028-30.  Back to cited text no. 9
    
10.
Mishra AK, Yadav P, Mishra A. A systemic review on staphylococcal scalded skin syndrome (SSSS): A rare and critical disease of neonates. Open Microbiol J 2016;10:150-9.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]



 

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