Nikolsky's sign

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Nikolsky's sign
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg
Differential diagnosis Stevens–Johnson syndrome

Nikolsky's sign is a clinical dermatological sign, named after Pyotr Nikolsky (1858–1940), a Russian physician who trained and worked in the Russian Empire. The sign is present when slight rubbing of the skin results in exfoliation of the outermost layer. [1] [2] [3] [4] A typical test would be to place the eraser of a pencil on the roof of a lesion and spin the pencil in a rolling motion between the thumb and forefinger. If the lesion is opened (i.e., skin sloughed off), then the Nikolsky's sign is present/positive.

Nikolsky's sign is almost always present in Stevens–Johnson syndrome/toxic epidermal necrolysis [5] and staphylococcal scalded skin syndrome, caused by the exfoliative toxin of Staphylococcus aureus . [1] It is also associated with pemphigus vulgaris and pemphigus foliaceus. [6] [7] It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin epidermal-epidermal junctions (e.g., desmosomes). The histological picture involves thinner, weaker attachments of the skin lesion itself to the normal skin – resulting in easier dislodgement.

The formation of new blisters upon slight pressure (direct Nikolsky) and shearing of the skin due to rubbing (indirect Nikolsky) is a sign of pemphigus vulgaris, albeit not a 100% reliable diagnosis. [8] In addition, another physical exam, the Asboe-Hansen signs, must be used to determine the absence of intracellular connections holding epidermal cells together. [9]

See also

Related Research Articles

Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN) overlap, they are considered febrile mucocutaneous drug reactions and probably part of the same spectrum of disease, with SJS being less severe. Erythema multiforme (EM) is generally considered a separate condition. Early symptoms of SJS include fever and flu-like symptoms. A few days later, the skin begins to blister and peel, forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia and multiple organ failure.

<span class="mw-page-title-main">Toxic epidermal necrolysis</span> Severe skin reaction involving fever, blistering, and peeling

Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, is a type of severe skin reaction. Together with Stevens–Johnson syndrome (SJS) it forms a spectrum of disease, with TEN being more severe. Early symptoms include fever and flu-like symptoms. A few days later the skin begins to blister and peel forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia, and multiple organ failure.

<span class="mw-page-title-main">Pemphigus</span> Blistering autoimmune diseases

Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root pemphix, meaning "blister".

<span class="mw-page-title-main">Staphylococcal scalded skin syndrome</span> Medical condition

Staphylococcal scalded skin syndrome (SSSS) is a dermatological condition caused by Staphylococcus aureus.

<span class="mw-page-title-main">Bullous pemphigoid</span> Autoimmune disease of skin and connective tissue characterized by large blisters

Bullous pemphigoid is an autoimmune pruritic skin disease that typically occurs in people aged over 60, that may involve the formation of blisters (bullae) in the space between the epidermal and dermal skin layers. It is classified as a type II hypersensitivity reaction, which involves formation of anti-hemidesmosome antibodies, causing a loss of keratinocytes to basement membrane adhesion.

<span class="mw-page-title-main">Pemphigus vulgaris</span> Chronic blistering skin disease

Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from the Greek word pemphix, meaning blister. It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. As desmosomes are attacked, the layers of skin separate and the clinical picture resembles a blister. These blisters are due to acantholysis, or breaking apart of intercellular connections through an autoantibody-mediated response. Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn.

<span class="mw-page-title-main">Desmoglein-1</span> Protein found in humans

Desmoglein-1 is a protein that in humans is encoded by the DSG1 gene. Desmoglein-1 is expressed everywhere in the skin epidermis, but mainly it is expressed in the superficial upper layers of the skin epidermis.

<span class="mw-page-title-main">Pemphigoid</span> Autoimmune blistering diseases

Pemphigoid is a group of rare autoimmune blistering diseases of the skin and mucous membranes. As its name indicates, pemphigoid is similar in general appearance to pemphigus, however unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.

Desquamative gingivitis is an erythematous (red), desquamatous (shedding) and ulcerated appearance of the gums. It is a descriptive term and can be caused by several different disorders.

Exfoliatin is a Staphylococcus aureus exotoxin that causes a blistering of the skin known as staphylococcal scalded skin syndrome, usually in infants.

The Asboe-Hansen sign refers to the extension of a large blister to adjacent unblistered skin when pressure is put on the top of it. It is seen along with Nikolsky's sign, both used to assess the severity of some blistering diseases such as pemphigus vulgaris and severe bullous drug reactions.

Pemphigus foliaceus is an autoimmune blistering disease of the skin. Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base. Mucosal involvement is absent even with widespread disease.

Pemphigus erythematosus is a localized form of pemphigus foliaceus with features of lupus erythematosus.

Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes.

<span class="mw-page-title-main">Generalized bullous fixed drug eruption</span> Medical condition

Generalized bullous fixed drug eruption (GBFDE) most commonly refers to a drug reaction in the erythema multiforme group. These are uncommon reactions to medications, with an incidence of 0.4 to 1.2 per million person-years for toxic epidermal necrolysis and 1.2 to 6.0 per million person-years for Stevens–Johnson syndrome. The primary skin lesions are large erythemas, most often irregularly distributed and of a characteristic purplish-livid color, at times with flaccid blisters.

Pyotr Vasilyevich Nikolsky was an Imperial Russian and later Soviet dermatologist. He was born in Usman, Tambov Governorate, Russian Empire and earned his medical degree from the Saint Vladimir Imperial University of Kyiv in 1884. After graduating, he studied under Mikhail Stukovenkov at the Department of Dermatology and Venerology in Kyiv. In 1896, he defended his doctoral thesis on pemphigus foliaceus, in which he described a dermatological condition involving a weakening relationship among the epidermal layers. The sloughing of skin associated with certain varieties of this condition is now referred to as Nikolsky's sign. In 1898, he became a professor at the Imperial University of Warsaw, and later established the Department of Dermatology and Venerology in Rostov at what is now Rostov State Medical University.

<span class="mw-page-title-main">Vesiculobullous disease</span> Presence of fluid-filled lesions in the skin or mucous membranes

A vesiculobullous disease is a type of mucocutaneous disease characterized by vesicles and bullae. Both vesicles and bullae are fluid-filled lesions, and they are distinguished by size. In the case of vesiculobullous diseases which are also immune disorders, the term immunobullous is sometimes used. Examples of vesiculobullous diseases include:

Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. It is one of the pemphigoid diseases that can result in scarring.

Autoimmune skin diseases occur when the immune system of an infected animal attacks its own skin. In dogs, autoimmune skin diseases are usually not detected until visible symptoms appear, which differs from detection in humans who are able to verbally express their concerns. Genetics, nutrition, and external environmental factors all collectively contribute to increasing the probability an autoimmune skin disease occurring. The severity of symptoms varies based on the specific disease present and how far it has progressed. Diagnosis often requires the onset of visible symptoms and for a biopsy to be performed. For many diseases, the condition itself cannot be cured, but a veterinarian can prescribe medications and other forms of treatment to help manage the symptoms of the dog.

References

  1. 1 2 Moss C, Gupta E (September 1998). "The Nikolsky sign in staphylococcal scalded skin syndrome". Arch. Dis. Child. 79 (3): 290. doi:10.1136/adc.79.3.290. PMC   1717681 . PMID   9875032.
  2. Schwartz, Robert A (2023-04-24). "Pemphigus Foliaceus: Practice Essentials, Background, Pathophysiology". Medscape Reference. Archived from the original on 2017-07-31. Retrieved 2024-08-08.
  3. Nikolski PV. Materiali K.uchenigu o pemphigus foliaceus [doctoral thesis]. Kiev. 1896.
  4. "MedilinePlus: Nikolsky's sign". Archived from the original on 2016-07-05. Retrieved 2018-03-21.
  5. Asz J, Asz D, Moushey R, Seigel J, Mallory SB, Foglia RP (December 2006). "Treatment of toxic epidermal necrolysis in a pediatric patient with a nanocrystalline silver dressing". J. Pediatr. Surg. 41 (12): e9–12. doi:10.1016/j.jpedsurg.2006.08.043. PMID   17161178.
  6. Ma, O. Emergency Medicine Manual. McGraw Hill. 2004.
  7. Beigi, Pooya Khan Mohammad (2018). "Background". A Clinician's Guide to Pemphigus Vulgaris. Springer, Cham. pp. 3–10. doi:10.1007/978-3-319-67759-0_1. ISBN   9783319677583.
  8. Venugopal, Supriya S.; Murrell, Dédée F. (July 2011). "Diagnosis and clinical features of pemphigus vulgaris". Dermatologic Clinics. 29 (3): 373–380, vii. doi:10.1016/j.det.2011.03.004. ISSN   1558-0520. PMID   21605802.
  9. Corwin, J (2016). "Pemphigus vulgaris. In: Ferri FF, editor". Ferri's Clinical Advisor: 945–6.