The Nikolsky sign in staphylococcal scalded skin syndrome
- CELIA MOSS, Consultant Dermatologist
- EMMA GUPTA, Senior House Officer in Paediatrics, Birmingham Children’s Hospital, UK
Editor,—Ladhani and Evans, in their account of staphylococcal scalded skin syndrome (SSSS),1accurately describe “large fluid-filled bullae which quickly rupture on slightest pressure” but incorrectly call this the Nikolsky sign. Fragility of blisters merely reflects their superficial position in the epidermis. The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin. The defect may be due to staphylococcal toxin as in SSSS, or to epidermal antibodies as in pemphigus. In his thesis on pemphigus in 1895, the Russian dermatologist Pyotr Vasilyevich Nikolsky (1858–1940) described “a weakening relationship and contact among the epidermal layers even in places between lesions on the seemingly unaffected skin”. He described three ways of eliciting the sign: (1) the stratum corneum, when pulled, can be stripped off over large areas, and it is possible to displace the stratum corneum of (2) healed skin and (3) healthy uninvolved skin by rubbing. Nikolsky himself gave some credit for determining this sign to his teacher Professor Stoukavenkow at the University of Kiev (1884–1935).2
Recently, a 14 month old girl under our care dramatically demonstrated the Nikolsky sign in SSSS. Three days after a minor skin injury on her left thumb which became infected, she developed irritability, coryza, and spreading erythema starting on the face and neck. She presented the next day with exquisitely tender red skin, and within 24 hours large areas of superficial epidermis separated, particularly under the edges of clothing, and in the axillae. Sheets of epidermis peeled back revealing painful raw areas (fig 1). Sparing of the mucosae excluded the alternative diagnoses of toxic epidermal necrolysis and Stevens-Johnson syndrome. She recovered promptly with intravenous flucloxacillin. As would be expected from the superficial level of the epidermal split in SSSS there was no scarring.