History

Fact Explanation
Rash followed by skin exfoliation. It is induced by the exfoliative toxins (ET) of Staphylococcus aureus. These toxins cause cleavage of desmoglein 1 complex, a protein in the desmosomes, which hold keratinocytes together in the zona granulosa of the epidermis. This leads to the formation of flaccid bullae, that rupture easily [1].
Fever and malaise. These symptoms occur as part of the systemic inflammatory response [2].
Skin tenderness. Epidermal rupture results in large sheets of raw, denuded skin which is painful [2].
Preceeding history of skin sepsis, sore throat, umbilical stump infection, etc. The disease is thought to arise from systemic ET absorption from these sites of staphylococcal infection followed by bloodstream diffusion to the cutaneous target [3].
References
  1. ARORA P, KALRA VK, RANE S, MCGRATH EJ, ZEGARRA-LINARES R, CHAWLA S. Staphylococcal scalded skin syndrome in a preterm newborn presenting within first 24 h of life BMJ Case Rep [online] :bcr0820114733 [viewed 04 August 2014] Available from: doi:10.1136/bcr.08.2011.4733
  2. MOCKENHAUPT MAJA, IDZKO MARCO, GROSBER MARTINE, SCHOPF ERWIN, NORGAUER JOHANNES. Epidemiology of Staphylococcal Scalded Skin Syndrome in Germany. J Invest Dermatol [online] 2005 April, 124(4):700-703 [viewed 04 August 2014] Available from: doi:10.1111/j.0022-202X.2005.23642.x
  3. YAMASAKI O., YAMAGUCHI T., SUGAI M., CHAPUIS-CELLIER C., ARNAUD F., VANDENESCH F., ETIENNE J., LINA G.. Clinical Manifestations of Staphylococcal Scalded-Skin Syndrome Depend on Serotypes of Exfoliative Toxins. Journal of Clinical Microbiology [online] December, 43(4):1890-1893 [viewed 05 August 2014] Available from: doi:10.1128/JCM.43.4.1890-1893.2005

Examination

Fact Explanation
Flaccid bullae and patchy, sheetlike exfoliation of skin. It is induced by the exfoliative toxins (ET) of Staphylococcus aureus. These toxins cause cleavage of desmoglein 1 complex, a protein in the desmosomes, which hold keratinocytes together in the zona granulosa of the epidermis. This leads to the formation of flaccid bullae, that rupture easily [1].
Nikolsky sign (gentle stroking of the skin causes the skin to separate at the epidermis). Due to a weakening relationship and contact among the epidermal layers even in places between lesions on the seemingly unaffected skin indicating a plane of cleavage in the skin caused by the ET [2].
Warmth and tenderness on palpation of skin. Epidermal rupture results in large sheets of raw, denuded and inflamed skin which is painful [3].
Dry mouth, dry eyes, loss of skin turgor and other features of dehydration. The resulting raw, denuded skin tends to cause extensive fluid loss [3].
References
  1. ARORA P, KALRA VK, RANE S, MCGRATH EJ, ZEGARRA-LINARES R, CHAWLA S. Staphylococcal scalded skin syndrome in a preterm newborn presenting within first 24 h of life BMJ Case Rep [online] :bcr0820114733 [viewed 04 August 2014] Available from: doi:10.1136/bcr.08.2011.4733
  2. MOSS C, GUPTA E. The Nikolsky sign in staphylococcal scalded skin syndrome Arch Dis Child [online] 1998 Sep, 79(3):290 [viewed 05 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1717681
  3. MOCKENHAUPT MAJA, IDZKO MARCO, GROSBER MARTINE, SCHOPF ERWIN, NORGAUER JOHANNES. Epidemiology of Staphylococcal Scalded Skin Syndrome in Germany. J Invest Dermatol [online] 2005 April, 124(4):700-703 [viewed 04 August 2014] Available from: doi:10.1111/j.0022-202X.2005.23642.x

Differential Diagnoses

Fact Explanation
Toxic epidermal necrolysis. It also presents with sheetlike loss of epidermis with flaccid blisters, but mucous membrane involvement also occurs [1].
Thermal burns. Painful skin blistering occurs with exposure to hot temperatures [2].
Pemphigus foliaceus. It presents with superficial skin erosions but the lesions are scaly and crusted [3].
References
  1. ROUJEAU JEAN-CLAUDE, CHOSIDOW OLIVIER, SAIAG PHILIPPE, GUILLAUME JEAN-CLAUDE. Toxic epidermal necrolysis (Lyell syndrome). Journal of the American Academy of Dermatology [online] 1990 December, 23(6):1039-1058 [viewed 05 August 2014] Available from: doi:10.1016/0190-9622(90)70333-D
  2. ORGILL DENNIS P.. Excision and Skin Grafting of Thermal Burns. N Engl J Med [online] 2009 February, 360(9):893-901 [viewed 05 August 2014] Available from: doi:10.1056/NEJMct0804451
  3. STANLEY JOHN R., AMAGAI MASAYUKI. Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded-Skin Syndrome. N Engl J Med [online] 2006 October, 355(17):1800-1810 [viewed 05 August 2014] Available from: doi:10.1056/NEJMra061111

Investigations - for Diagnosis

Fact Explanation
Elevated white blood cell count. It occurs as part of the systemic inflammatory response to staphylococcal infection and associated toxin production [1].
Elevated erythrocyte sedimentation rate. It occurs as part of the systemic inflammatory response to staphylococcal infection and associated toxin production [1].
Positive gram stain/ culture for Staphylococci at remote infection site. The disease is thought to arise from systemic ET absorption from these sites of staphylococcal infection followed by bloodstream diffusion to the cutaneous target [2].
References
  1. MOCKENHAUPT MAJA, IDZKO MARCO, GROSBER MARTINE, SCHOPF ERWIN, NORGAUER JOHANNES. Epidemiology of Staphylococcal Scalded Skin Syndrome in Germany. J Invest Dermatol [online] 2005 April, 124(4):700-703 [viewed 04 August 2014] Available from: doi:10.1111/j.0022-202X.2005.23642.x
  2. YAMASAKI O., YAMAGUCHI T., SUGAI M., CHAPUIS-CELLIER C., ARNAUD F., VANDENESCH F., ETIENNE J., LINA G.. Clinical Manifestations of Staphylococcal Scalded-Skin Syndrome Depend on Serotypes of Exfoliative Toxins. Journal of Clinical Microbiology [online] December, 43(4):1890-1893 [viewed 05 August 2014] Available from: doi:10.1128/JCM.43.4.1890-1893.2005

Investigations - Screening/Staging

Fact Explanation
Serum electrolytes and renal function tests. The resulting raw, denuded skin tends to cause extensive fluid loss and dehydration [1].
References
  1. MOCKENHAUPT MAJA, IDZKO MARCO, GROSBER MARTINE, SCHOPF ERWIN, NORGAUER JOHANNES. Epidemiology of Staphylococcal Scalded Skin Syndrome in Germany. J Invest Dermatol [online] 2005 April, 124(4):700-703 [viewed 04 August 2014] Available from: doi:10.1111/j.0022-202X.2005.23642.x

Management - General Measures

Fact Explanation
Antipyretics. Eg: paracetomol 10-15 mg/kg 6 hourly. To combat the fever occurring as a result of the systemic inflammatory response [1].
Fluid rehydration. Lactated Ringer solution at 20 mL/kg initial bolus. Repeat the initial bolus, as clinically indicated, followed by maintenance therapy. The resulting raw, denuded skin tends to cause extensive fluid loss and dehydration [1].
References
  1. MOCKENHAUPT MAJA, IDZKO MARCO, GROSBER MARTINE, SCHOPF ERWIN, NORGAUER JOHANNES. Epidemiology of Staphylococcal Scalded Skin Syndrome in Germany. J Invest Dermatol [online] 2005 April, 124(4):700-703 [viewed 04 August 2014] Available from: doi:10.1111/j.0022-202X.2005.23642.x

Management - Specific Treatments

Fact Explanation
Topical therapy with fusidic acid or mupirocin. Organisms may be present in the lesions [1].
Nafcillin. Intravenous or intramuscular administration. 100-200 mg/kg/day in divided doses. It is a penicillinase-resistant semisynthetic penicillin derivative [2]. Therefore it is the drug of choice when the susceptibility of the organism is not yet known.
Vancomycin. Intravenous or intramuscular administration. 40 mg/kg/day in divided doses. It has coverage against methicillin-resistant Staphylococcus aureus (MRSA) [3].Therefore it is given to those who initially appear toxic or who did not respond to nafcillin.
References
  1. LADHANI S. Recent developments in staphylococcal scalded skin syndrome. Clin Microbiol Infect [online] 2001 June, 7(6):301-307 [viewed 05 August 2014] Available from: doi:10.1046/j.1198-743x.2001.00258.x
  2. RUIZ D. E., WARNER J. F.. Nafcillin Treatment of Staphylococcus aureus Meningitis. Antimicrobial Agents and Chemotherapy [online] 1976 March, 9(3):554-555 [viewed 06 August 2014] Available from: doi:10.1128/AAC.9.3.554
  3. SCHWEIZER MARIN L, FURUNO JON P, HARRIS ANTHONY D, JOHNSON J KRISTIE, SHARDELL MICHELLE D, MCGREGOR JESSINA C, THOM KERRI A, COSGROVE SARA E, SAKOULAS GEORGE, PERENCEVICH ELI N. Comparative effectiveness of nafcillin or cefazolin versus vancomycin in methicillin-susceptible Staphylococcus aureus bacteremia. Array [online] 2011 December [viewed 06 August 2014] Available from: doi:10.1186/1471-2334-11-279