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Nikolskys Sign

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101. Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

of epithelium slide off the entire body at pressure points (Nikolsky sign), exposing weepy, painful, and erythematous skin. Painful oral crusts and erosions, keratoconjunctivitis, and genital problems (eg, urethritis, phimosis, vaginal synechiae) accompany skin sloughing in up to 90% of cases. Bronchial epithelium may also slough, causing cough, dyspnea, pneumonia, pulmonary edema, and hypoxemia. Glomerulonephritis and hepatitis may develop. Diagnosis Clinical evaluation Often skin biopsy Diagnosis is often (...) (showing necrotic epithelium) if clinical characteristics (eg, target lesions progressing to bullae, ocular and mucous membrane involvement, Nikolsky sign, desquamation in sheets) are inconclusive. Early treatment decreases the often high mortality rate. Except for mild cases, treat SJS/TEN in a burn unit and with intensive supportive care. Consult ophthalmology if the eyes are affected. Consider cyclosporine and possibly plasma exchange for severe cases. Last full review/revision May 2017 by Mercedes

2013 Merck Manual (19th Edition)

102. Stomatitis

for the location and nature of any lesions. The skin and other mucosal surfaces (including the genitals) are inspected for any lesions, rash, petechiae, or desquamation. Any bullous lesions are rubbed for the Nikolsky sign (upper layers of epidermis move laterally with slight pressure or rubbing of skin adjacent to a blister). Red flags The following findings are of particular concern: Fever Cutaneous bullae Ocular inflammation Immunocompromise Interpretation of findings Occasionally, causes are obvious (...) skin. The Nikolsky sign is usually positive in SJS and pemphigus vulgaris. Cutaneous vesicles are typical with chickenpox or herpes zoster (see ). Unilateral lesions in a band along a dermatome suggest herpes zoster. Diffuse, scattered vesicular and pustular lesions in different stages suggest chickenpox. usually has a macular rash, desquamation of hands and feet, and conjunctivitis; it occurs in children, usually those < 5 yr. Oral findings include erythema of the lips and oral mucosa. Other

2013 Merck Manual (19th Edition)

103. Bullous Pemphigoid

peripheral vesicles, can occur. Rarely, small blisters develop on the mucosa. Leukocytosis and eosinophilia are common, but fever is rare. The Nikolsky sign, where upper layers of epidermis move laterally with slight pressure or rubbing of skin adjacent to a blister, is negative. Diagnosis Skin biopsy and IgG titers If blisters develop, bullous pemphigoid needs to be differentiated from , a blistering disorder with a worse prognosis; differentiation is usually possible using clinical criteria (see Table (...) : ). Table Distinguishing Bullous Pemphigoid From Pemphigus Vulgaris Disorder Appearance of Lesion Oral Involvement Itching Nikolsky Sign Prognosis Bullous pemphigoid Tense bullae that developed on normal-appearing or erythematous skin or urticarial-appearing Rare, with small blisters Common Generally negative Usually good; occasionally fatal in the elderly Pemphigus vulgaris Flaccid bullae of various sizes Often shearing off of skin or mucosa, leaving painful erosions Typically starts in the mouth

2013 Merck Manual (19th Edition)

104. Staphylococcal Scalded Skin Syndrome

in areas of friction, such as intertriginous areas, buttocks, hands, and feet. Intact blisters extend laterally with gentle pressure (Nikolsky sign). The epidermis may peel easily, often in large sheets. Widespread desquamation occurs within 36 to 72 h, and patients become very ill with systemic manifestations (eg, malaise, chills, fever). Desquamated areas appear scalded. Loss of the protective skin barrier can lead to and to fluid and electrolyte imbalance. Diagnosis Biopsy Cultures from areas (...) (also called epidermolysin), a toxin that splits the upper part of the epidermis just beneath the granular cell layer by targeting desmoglein-1 (see also ). The primary infection often begins during the first few days of life in the umbilical stump or diaper area; in older children, the face is the typical site. Toxin produced in these areas enters the circulation and affects the entire skin. Symptoms and Signs Staphylococcal Scalded Skin Syndrome Image courtesy of Thomas Habif, MD. Staphylococcal

2013 Merck Manual (19th Edition)

105. Description of Skin Lesions

pressure (eg, stroking or scratching the skin) in the distribution of the pressure. Up to 5% of normal patients may exhibit this sign, which is a form of physical urticaria. Darier sign refers to rapid swelling of a lesion when stroked. It occurs in patients with urticaria pigmentosa or . Nikolsky sign is epidermal shearing that occurs with gentle lateral pressure on seemingly uninvolved skin in patients with and some autoimmune . Auspitz sign is the appearance of pinpoint bleeding after scale (...) of drugs or metals in the skin, including minocycline , amiodarone , and silver (argyria). Ischemic skin appears purple to gray in color. Deep dermal nevi appear blue. Black skin lesions may be melanocytic, including nevi and . Black eschars are collections of dead skin that can arise from infarction, which may be caused by infection (eg, , angioinvasive fungi including Rhizopus, ), , arterial insufficiency, or . Other Clinical Signs Dermatographism is the appearance of an urticarial wheal after focal

2013 Merck Manual (19th Edition)

106. Pemphigus Vulgaris

Pemphigus vulgaris should be suspected in patients with unexplained chronic mucosal ulceration, particularly if they have bullous skin lesions. This disorder must be differentiated from other disorders that cause chronic oral ulcers and from other bullous dermatoses (eg, , , , , , , , bullous ). Two clinical findings, both reflecting lack of epidermal cohesion, that are somewhat specific for pemphigus vulgaris are the following: Nikolsky sign: Upper layers of epidermis move laterally with slight (...) reference 1. : Rituximab used as a first-line single agent in the treatment of pemphigus vulgaris. J Am Acad Dermatol 65 (5):1064–1065, 2011. doi: 10.1016/j.jaad.2010.06.033. Key Points About half of patients with pemphigus vulgaris have only oral lesions. Use Nikolsky and Asboe-Hansen signs to help clinically differentiate pemphigus vulgaris from other bullous disorders. Confirm the diagnosis by immunofluorescence testing of skin samples. Treat with systemic corticosteroids, with or without other

2013 Merck Manual (19th Edition)

107. Rash in Infants and Young Children

erythema that develop large, flaccid blisters, which are easily ruptured, leaving large areas of desquamation Lateral extension of blisters with gentle pressure (positive Nikolsky sign) Spares the mucous membranes Usually in children < 5=""> Clinical evaluation Sometimes confirmed by biopsy and/or cultures Scaly, oval lesions with a slightly raised border and central clearing Mild itching Clinical evaluation Sometimes scrapings of lesions for KOH wet mount Viral infection (systemic) Maculopapular rash (...) in the mouth and lips but sometimes in the genital and anal regions Widespread areas of painful erythema that develop large, flaccid blisters, which are easily ruptured, leaving large areas of desquamation; possibly affecting the soles but usually not the scalp Lateral extension of blisters with gentle pressure (positive Nikolsky sign) Sometimes use of a causative drug (eg, sulfonamides, penicillins, anticonvulsants) Clinical evaluation Sometimes biopsy Well-circumscribed, pruritic, red, raised lesions

2013 Merck Manual (19th Edition)

108. Novel Approaches in Preventing and Limiting Events III Trial (NAPLES III): Bivalirudin in High-risk Bleeding Patients

of this drug. Bivalirudin (The Medicine's Co., Parsippany, NJ) is a synthetic direct thrombin inhibitor approved for patients with stable and unstable coronary syndromes undergoing PCI. Favourable properties of bivalirudin may minimize bleeding. Several clinical and procedural factors have been evaluated to identify patients exposed to a higher risk of hemorrhages. Nikolsky et al. have developed a risk score (validated on REPLACE-1 and REPLACE-2 data) based on clinical variable useful to predict (...) : All Accepts Healthy Volunteers: No Criteria Inclusion Criteria: • Male or female able to understand and sign a witnessed informed consent Age ≥ 18 ys Patients with stable (CCS 1-4) or unstable angina pectoris (but with the most recent anginal episode occurring >48 hours before the procedure) or documented silent ischemia Stable Hemodynamic conditions (systolic BP > 100 HR > 40 < 100). No clinical and ECG changes suggestive of ongoing acute or recent (<48 hours) myocardial infarction. Bleeding risk

2011 Clinical Trials

109. Evaluating Sirolimus to Treat Autoimmune Blistering Dermatosis Pemphigus

of age or older. Subject must have an established diagnosis of pemphigus disorder via biopsy and/or serologic titer, as determined appropriate by the lead researcher. Subject must have active disease at the time of enrollment, as defined by a positive Nikolsky sign. Subject must not be taking any immunosuppressive medication or therapy other than corticosteroids. Subject must be able to understand and follow directions. If female, subject is not currently breast feeding and/or pregnant as confirmed

2011 Clinical Trials

110. Fatal toxic epidermal necrolysis associated with minoxidil. (Abstract)

for a cerebrovascular accident and uncontrolled hypertension. On hospital day 12, oral minoxidil was added to her drug regimen. On day 23, she developed a maculopapular rash on her face that gradually diffused to her chest and back. Vesicles and papular lesions extended to her extremities and mucosal membranes; results of a skin biopsy revealed SJS. A positive Nikolsky's sign (blisters spread on application of pressure) was detected. On days 27-31, diffuse bullae developed with rash exacerbation. Skin detachment

2009 Pharmacotherapy

111. Prevention of Contrast-Induced Nephropathy in the Emergency Department

injection may have important implications for the risk of contrast-induced nephropathy. Arteriography generally requires larger contrast volumes than CT scans, which increases the probability of nephropathy. x 9 Nikolsky, E., Mehran, R., Turcot, D. et al. Impact of chronic kidney disease on prognosis of patients with diabetes mellitus treated with percutaneous coronary intervention. Am J Cardiol. 2004 ; 94 : 300–305 | | | | | In addition, this higher risk of contrast-induced nephropathy is compounded

2007 Evidence-Based Emergency Medicine

112. Amniotic membrane transplantation in acute phase of toxic epidermal necrolysis with severe corneal involvement. (Abstract)

Amniotic membrane transplantation in acute phase of toxic epidermal necrolysis with severe corneal involvement. To report successful management of acute stage toxic epidermal necrolysis (TEN) by amniotic membrane transplantation.Interventional case report. METHOD/INTERVENTION: A 6-year-old boy who had convulsions and fever due to encephalitis was treated by oral phenobarbital. Two weeks later, he developed a high fever and skin rashes involving >40% of the body, with a positive Nikolsky sign

2006 Ophthalmology

113. Increased activity of plasma and tissue kallikreins, plasma kininase II and salivary kallikrein in pemphigus foliaceus (fogo selvagem). (Abstract)

-weight kininogen (HK) and low-molecular-weight kininogen (LK), and the activity of plasma kallikrein, tissue kallikrein and kininase II in plasma of patients with PF presenting with Nikolsky's sign. As kidneys and salivary glands are relevant sources of tissue kallikrein for plasma, we also evaluated urinary/salivary kallikrein and urinary kininase II activities.Fifteen patients and 15 age- and sex-matched controls were studied. Kininogen levels were determined by enzyme-linked immunosorbent assay

2005 British Journal of Dermatology

114. Toxic Epidermal Necrolysis

form and then coalesce. They generally increase in number over 3-4 days (sometimes hours). The epidermis can then slough in sheets. There may be hyperpyrexia. Hypotension and tachycardia can develop secondary to dehydration and hypovolaemia. Nikolsky's sign may be positive: if areas of seemingly normal skin between lesions are rubbed, the epidermis easily separates from its underlying surface. Differential diagnosis . (chemical, light or heat). . , (generally of slower onset). . . (desquamation

2008 Mentor

115. Stevens-Johnson Syndrome

pathognomonic. Lesions may become bullous and later rupture. The skin becomes susceptible to secondary infection. Urticarial lesions are usually not pruritic. Nikolsky sign is positive (mechanical pressure to skin leading to blistering within minutes or hours). Mucosal involvement: erythema, oedema, sloughing, blistering, ulceration and necrolysis. Eye: conjunctivitis, corneal ulcerations. Genital: erosive vulvovaginitis or balanitis. Differential diagnosis Acute generalised exanthematous pustulosis (...) and diarrhoea, and malaise. Mucocutaneous lesions develop suddenly and clusters of outbreaks last from 2-4 weeks. The lesions are usually not pruritic. Mouth: severe oromucosal ulceration. Respiratory involvement may cause a cough productive of a thick purulent sputum. Patients with genitourinary involvement may complain of dysuria or an inability to pass urine. Ocular symptoms: painful red eye, purulent conjunctivitis, photophobia, blepharitis. Signs General examination: fever, tachycardia, hypotension

2008 Mentor

116. Dermatological Descriptive Terms

of periodontal disease that affects the gums. Miscellaneous terms In Köbner's phenomenon, lesions arise in an area of trauma. This is typical of psoriasis and lichen planus. Dermatographism (dermatographia) is the ability to write on skin. It occurs with urticaria. If a firm instrument, like an orange stick, is used to make lines or letters on the skin then, shortly afterwards, the wheal will form and the pattern of marks will be very obvious. In Nikolsky's sign, the skin of a bulla is lightly rubbed

2008 Mentor

117. A new type of staphylococcal exfoliative toxin from a Staphylococcus aureus strain isolated from a horse with phlegmon. Full Text available with Trip Pro

gel electrophoresis (7.5% polyacrylamide). The new sET elicited general exfoliation of the epidermis with the so-called Nikolsky sign when inoculated into both 3-day-old mice and 1-day-old chicks, whereas sETA and sETB from human strains of S. aureus caused exfoliation in a 3-day-old mouse alone and shET from a porcine strain of Staphylococcus hyicus caused exfoliation in 1-day-old chicks alone. Intraepidermal splitting was observed at the granular layer of the epidermis of mice inoculated

1994 Infection and immunity

118. New Exfoliative Toxin Produced by a Plasmid-Carrying Strain of Staphylococcus hyicus Full Text available with Trip Pro

SHET caused exfoliation of the epidermis as determined by the so-called Nikolsky sign when inoculated into 1-day-old chickens. The new SHET was serologically different from Staphylococcus aureus exfoliative toxins (ETs) (ETA, ETB, and ETC) and from the SHET from the plasmidless strain but showed the same molecular weight as the other serotypes of toxins on SDS-PAGE. It was thermolabile and lost its toxicity after being heated at 60 degrees C for 30 min. We propose that the new SHET be designated

1999 Infection and immunity

119. The Nikolsky sign in staphylococcal scalded skin syndrome Full Text available with Trip Pro

The Nikolsky sign in staphylococcal scalded skin syndrome 9875032 1999 01 12 2008 11 20 1468-2044 79 3 1998 Sep Archives of disease in childhood Arch. Dis. Child. The Nikolsky sign in staphylococcal scalded skin syndrome. 290 Moss C C Gupta E E eng Case Reports Comment Letter England Arch Dis Child 0372434 0003-9888 AIM IM Arch Dis Child. 1998 Jan;78(1):85-8 9534685 Epidermis pathology Female Humans Infant Staphylococcal Scalded Skin Syndrome diagnosis pathology Thumb injuries 1999 1 6 1999 1 6

1998 Archives of Disease in Childhood

120. Microscopic Nikolsky's sign. (Abstract)

Microscopic Nikolsky's sign. Nikolsky's sign is a useful clinical sign in patients with active pemphigus. It does not however, indicate the level of split in the skin, and is seldom present in partially-treated patients. The purpose of this study was two fold: (i) to document the microscopic or subclinical counterpart of clinical Nikolsky's sign; and (ii) to improve the diagnostic yield of routine histopathology in pemphigus patients in whom the lesions are infected/old and are therefore

1999 Clinical and experimental dermatology Controlled trial quality: uncertain

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