Dermatologic Manifestations of Staphylococcal Scalded Skin Syndrome Clinical Presentation

Updated: Apr 25, 2017
  • Author: Elizabeth U Rogozinski, MD, MS; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Staphylococcal scalded skin syndrome (SSSS) originates from a focus of infection that may be a purulent conjunctivitis, otitis media, or occult nasopharyngeal infection. A case of SSSS from a nasal septal abscess, presumably arising from a traumatic hematoma (a tsunami survivor), has been reported, as well as adult infection in a previously healthy man following a dental procedure. [25, 27] In premature infants, a previous amniocentesis procedure and intrauterine infection have been reported as triggers for SSSS, although reports of outbreaks within nurseries is well described and should still be considered in premature infants in addition to all term infants. [28, 29]

Circumcision sites and the umbilicus are other common niduses of infection in the newborn period. SSSS usually begins with fever, irritability, and a generalized, faint, orange-red, macular erythema with cutaneous tenderness. Irritability in children is very commonly reported. Children may complain of vague pain in regions of developing lesions, such as abdominal pain with a superficial abdominal lesion.

SSSS can present with immediate widespread erythema or may begin with a localized site of exudative erythema and rapidly spread, usually within hours. An area of local, painful bullae that remains localized over days is unlikely to be SSSS and is more likely to be bullous impetigo. These lesions should be treated appropriately, but monitored closely as there have been reports of misdiagnosis, missing SSSS completely, and bullous impetigo evolving into SSSS. [30, 31]

Periorificial and flexural accentuation may be observed. A positive Nikolsky sign (separation of the superficial layer of the epithelium on gentle pressure) can often be elicited at this stage.

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Physical Examination

Patients usually present with fever and pain associated with a generalized, macular, salmon-colored to bright-red rash, as shown in the image below. Within 24-48 hours, the rash progresses from a scarlatiniform rash to an edematous, blistering eruption.

An infant with characteristic coloring of the skin An infant with characteristic coloring of the skin that looks as though the child has been scalded. Exfoliation of thin sheets of skin is present.

Characteristic tissue paper–like wrinkling of the epidermis is followed by the appearance of large, flaccid bullae in the axillae, in the groin, and around the body orifices. Perioral involvement is commonly seen in children and adults. Periumbilical and perineal involvement is more common in infants. Extremity involvement is seen more often in older children. The Nikolsky sign, the sloughing of superficial epidermis with gentle shearing force applied to the top of a lesion, is present in staphylococcal scalded skin syndrome (SSSS). See the image below.

Staphylococcal scalded skin syndrome in an adult. Staphylococcal scalded skin syndrome in an adult.

Subsequent generalized involvement occurs elsewhere on the body, but infection spares the mucous membranes, owing to the presence of unaffected Dsg-3 throughout all strata of the mucosa.

As sheets of epidermis are shed, a moist erythematous base is revealed. Patients experience pain throughout the evolution of the disease owing to the intact free nerve endings terminating in the epidermis. Twenty-four hours following the exfoliation of the skin, the underlying epidermis dries, resulting in a shiny crust. With drying, perioral and periorbital lesions may fissure.

Despite the dramatic clinical picture, the entire process usually subsides with superficial desquamation if antibiotics are administered. Healing is typically rapid, with complete resolution within 5-7 days, although 2-3 weeks is more commonly expected. Children may undergo a second phase of desquamation around day 10, but the disease process still completely resolves around day 14.

In adults, SSSS is frequently followed by bacteremia and pneumonia, favoring a poor prognosis.

Cultures obtained from intact bullae usually are sterile; this finding is consistent with hematogenous dissemination of a toxin produced at a distant focus of staphylococcal infection. Blood cultures usually are negative in infants and children, but commonly positive in adults.

An abortive form of SSSS, known as the scarlatiniform variant, shows the early erythrodermic and final desquamative stages seen in SSSS, but the bullous stage does not occur.

Other intermediate forms of scalded skin syndrome begin as localized bullous impetigo, but they evolve to produce regionally limited bullae and denuded areas that may or may not harbor staphylococci.

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Causes

See Pathophysiology.

ET-A and ET-B are atypical glutamic acid–specific serine proteases that specifically target Dsg-1. They are also sources of superantigenic activity and activate macrophages to produce proinflammatory cytokines, such as tumor necrosis factor-alpha and IL-6, and keratinocytes to produce IL-6. ET-A with an active site mutation was shown to possess similar inflammatory activity, indicating that the inflammatory activity of ET-A was separate from its epidermolytic activity, although it has not been shown to directly modulate cytokine release and may likely be due to a downstream effect after Dsg-1 cleavage. [5, 32] The preferential production of IL-6 from keratinocytes in response to ET-A activity results in localized immune dysregulation and lack of a significant WBC infiltrate. Systemic symptoms, particularly the characteristic rash, are very likely a direct result of the toxin.

A small number of patients with staphylococcal scalded skin syndrome (SSSS) develop low titers of immunoglobulin G antibodies specific for Dsg-1 after binding and systemic digestion of Dsg-1 by staphylococcal exfoliative toxins. [33] The relevance of this finding to the onset of autoimmune diseases remains to be proven. Other genetic or environmental factors may be needed to propagate production of pathogenic antibodies and to produce overt clinical pemphigus foliaceus. [34]

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Complications

Cellulitis, sepsis, and pneumonia are possible complications that may occur in children with staphylococcal scalded skin syndrome (SSSS). Cellulitis occurs after spreading of the infection to deeper layers of the skin. Dsg-1 is present in the deeper layers of the skin, and while Dsg-3 protects sloughing of the deeper layers, the effects of ET-A and ET-B create a weakened stratum spinosum, leading to the potential for cellulitis. Sepsis is the most serious complication and is the most common cause of mortality in neonates with SSSS.

Dehydration and secondary infection due to loss of the superficial layer of the skin are significant causes of morbidity and are critical issues early on in the disease process. Considerable electrolyte disturbances may ensue if dehydration is not managed at the first sign of disease.

Scarring is not seen in SSSS, owing to the sparing of the deeper layers of the skin.

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