From the Guidelines
The Nikolsky sign is a clinical finding where the top layer of skin (epidermis) separates from the underlying layers when gentle pressure is applied to the skin surface, and it is considered positive when lateral pressure on the skin causes the outer layer to slide away from the lower layers, resulting in blistering or peeling, as described in the British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018 1. This sign is primarily used to diagnose certain serious skin conditions, particularly autoimmune blistering disorders like pemphigus vulgaris, Stevens-Johnson syndrome, toxic epidermal necrolysis, and staphylococcal scalded skin syndrome. Some key points about the Nikolsky sign include:
- It is a helpful clinical indicator of epidermal necrolysis, although not specific for SJS/TEN, as it can also be positive in pemphigus 1
- The sign is positive if gentle lateral pressure on the skin causes the outer layer to slide away from the lower layers, resulting in blistering or peeling
- To test for the Nikolsky sign, a physician gently rubs or applies lateral pressure to an area of apparently normal skin near a lesion or blister
- The underlying mechanism involves the disruption of cell-to-cell adhesion in the epidermis, often due to autoantibodies targeting proteins that normally hold skin cells together
- A positive Nikolsky sign generally indicates a more severe skin condition that requires prompt medical attention and often specialized dermatological care. The most recent and highest quality study, the British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018 1, provides the best evidence for the definition and clinical significance of the Nikolsky sign.
From the Research
Definition of Nikolski Sign
- The Nikolski sign refers to the ability to induce peripheral extension of a blister as a consequence of applying lateral pressure to the border of an intact blister 2.
- It is a mechanical symptom that can be helpful in the diagnosis of certain skin diseases, including pemphigus 3.
Clinical Significance
- A positive Nikolski sign can be seen in other bullous diseases such as toxic epidermal necrolysis and staphylococcus scalded skin syndrome 2, 4, 5.
- The sign is characterized by the presence of cutaneous tenderness and a positive Nikolski sign, even prior to frank exfoliation 5.
- The Nikolski sign offers a moderately sensitive but highly specific tool for the diagnosis of pemphigus 3.