Positive Nikolsky Sign due to Staphylococcal Scaled Skin Syndrome

Figure 2. The patient 7 days after the diagnosis of SSSS. A 16-month-old previously healthy girl was admitted for acute onset of a tender, diffuse, erythematous, and exfoliating rash that was began on the neck and axilla and then spread to the face, trunk, genitalia, and extremities. The patient had increasing pain when she was touched or held, and her skin began to peel hours before admission. The examining physician elicited a positive Nikolsky sign; exfoliation affected 70% of the total body surface (Figure 1). There were no clinical signs of infection. Staphylococcus aureus was isolated from nasal and pharyngeal cultures. Investigation for exfoliative toxin gene expression by polymerase chain reaction was positive for exfoliative toxin B, confirming the diagnosis of staphylococcal scalded skin syndrome (SSSS). After a course of antibiotic therapy, the patient recovered completely within 15 days without scarring (Figure 2). Nikolsky sign is present when lateral traction of the skin leads to sloughing. Although the differential diagnosis includes pemphigus vulgaris, empidermolysis bullosa, secondand third-degree burns, the most common cause is exfoliative toxin-producing S aureus (causing bullous impetigo, Lyell disease, Ritter disease, or SSSS), and the clinical scenario is usually compelling. Toxic epidermal necrolysis also can cause a positive Nikolsky sign; this disorder occurs more frequently in older individuals and as an adverse drug effect. In SSSS, exfoliative toxin is disseminated hematogenously, targets the protein desmoglein I in the zona granulosa of the epidermis, and causes superficial cleavage of tissue with blistering. Clinical staphylococcal infection may or may not be present. Most cases of SSSS occur in