Dx: Staphylococcal scalded skin syndrome
Based on the patient’s classic presentation and exam findings, the physician suspected staphylococcal scalded skin syndrome. SSSS is a rare but serious condition that progresses quickly with high fevers and diffuse painful erythema. The exact epidemiology of SSSS is unclear; some articles report incidences between 0.09 and 0.13 cases per 1 million people.1 The mortality rate is about 5% due to complications of sepsis, superinfection, and electrolyte disturbances.2
SSSS is caused by Staphylococcus aureus from a localized source that produces exfoliative toxins A and B that spread hematogenously, causing extensive epidermal damage. Exotoxins bind to desmosomes, causing skin cells to lose adherence.3 Histopathology shows intraepidermal cleavage through the stratum granulosum.
Infants and younger children appear more likely to be affected by SSSS, although it may occur in older children or adults who are immunocompromised. It may be that younger children are most susceptible due to a lack of antibodies to the toxin produced or because of a delayed clearance of the toxin-antibody complex from an immature renal system.
What you’ll see. Patients with SSSS may have a prodrome of irritability, malaise, and fever. The rash is first noticeable as erythema in the flexural areas.4 The erythematous tender patches spread and coalesce into a scarlatiniform erythema. Fragile bullae become large sheets of epidermis that slough (a positive Nikolsky’s sign).5 The desquamated areas can exhibit a scalded appearance.3
Differential diagnosis includes TEN and SJS
There is a broad differential for vesiculobullous rashes, ranging from self-limiting conditions to those that are life threatening.
Toxic epidermal necrolysis (TEN), Stevens-Johnson Syndrome (SJS), and erythema multiforme major (EMM) are immunological reactions to certain drugs or infections varying in the severity of their presentation. EMM, SJS, and TEN involve the mucosal surfaces, while SSSS does not. The histopathology of these conditions also differs from SSSS as they have keratinocyte necrosis of varying levels of the skin, whereas SSSS only involves the epidermis.
SSSS also may be confused with drug reactions, such as DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome. DRESS typically is associated with anticonvulsants and sulfonamides and may have peripheral eosinophilia and a transaminitis.4
Continue to: Other more self-limited vesiculobullous rashes...