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Scalded Skin Syndrome

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There were multiple conditions on the differential, but ultimately a skin biopsy confirmed a diagnosis of Staphylococcal Scalded Skin Syndrome (SSSS). SSSS is a syndrome of acute exfoliation of the skin typically following an erythematous cellulitis. The severity varies from a few blisters localized to the site of infection to a severe exfoliation affecting almost the entire body. SSSS is caused by epidermolytic toxins produced by certain strains (5%) of Staphylococci and is usually seen in neonates and young children. The toxins act at the zona granulosa of the epidermis causing cleavage of desmoglein 1 complex, which is an important protein in desmosomes that anchor keratinocytes to each other. This results in the formation of fragile, tense …show more content…

Then there is generalized erythema rapidly followed by the development of flaccid blisters and desquamation, as seen in this patient. The mucous membranes are not involved, which is also consistent with our patient. The surrounding areas of her face were involved, but the mucous membranes were spared. This condition is also associated with a positive Nikolsky sign. A Nikolsky sign is the ability to extend the area of superficial sloughing by applying gentle lateral pressure on the surface of the skin at an apparently uninvolved site. This was found incidentally in our patient when the adhesive tape of an IV line was removed resulting in sloughing off of the skin below it. Furthermore, due to the sloughing off of skin with pressure, there tends to be increased desquamation in areas of mechanical stress like the flexural areas, buttocks, hands, and feet. If SSSS is suspected, cultures should be obtained from the blood, the urine, the nasopharynx, the perianal area, and any other abnormal skin or suspected focus of infection. The intact bullae are sterile and will come back without growth. In this patient, blood cultures had no growth to date and the pan-cultures from the mouth, nares, eyes, and anus were non-specific. Diagnosis for SSSS is usually clinical, although it may be confirmed with skin biopsy that shows a cleavage plane in the lower stratum …show more content…

SJS and TEN are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis. The two conditions are distinguished based on severity, which is scaled by evaluating the percentage of body surface area involved in the blisters and erosions. SJS is the less severe and more common condition with 30% of the body surface with mucous membranes involved in the majority of cases. The mucous membranes were not involved in this patient making SJS and TEN less likely. However, the mortality rate for SJS is 10% and TEN is 30%, which is why it was important to rule out these conditions before sending the patient home. Although SJS and TEN are usually triggered by medications, one study showed that approximately 20-25% of pediatric cases could not be clearly attributed to a drug. While this patient’s history does not indicate any new medications, we cannot simply rule out the possibility of SJS or TEN for that

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