2.15.10 Tichy Blistering Skin Diseases

8/2/2019 2.15.10 Tichy Blistering Skin Diseases

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Blistering Skin Eruptions

Jill Tichy, PGY III

February 15th, 2010


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Causes of Vesicles/Bullae

Primary Cutaneous Disease: Pemphigus, BullousPemphigus, Dermatitis Herpatiformis, ContactDermatitis, Erythema Multiforme, Stevens-Johnsonsyndrome, Toxic Epidermal Necrolysis, VZV, HSZ,Hand-foot-and-mouth disease, Staphylococcalscalded-skin syndrome, Scarlet Fever, Toxic Shock

Syndrome, Exfoliative Erythroderma Syndrome Systemic Diseases: Paraneoplastic pemphigus,

Porphyria Cutanea Tarda, Porphyria Variegata


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Nikolskys Sign

Staphylococcal Scalded Skin Syndrome

SJS/TENS

Positive when slight rubbing of the skin results inexfoliation of the skin's outermost layer

A "positive" Nikolsky's sign is associated withpemphigus vulgaris.

Nikolsky's sign is useful in differentiating betweenpemphigus vulgaris (where it is present or positive)and bullous pemphigoid (where it is absent)
http://en.wikipedia.org/wiki/Pemphigus_vulgarishttp://en.wikipedia.org/wiki/Bullous_pemphigoidhttp://en.wikipedia.org/wiki/Bullous_pemphigoidhttp://en.wikipedia.org/wiki/Pemphigus_vulgaris


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Toxic Epidermal Necrolysis

Bullae that arise on the widespread areas oferythema and then slough

The result is large areas of denuded skin

Sepsis and Respiratory Failure

Involvement of mucous membranes and

intestinal tract Drugs are primary offenders (95%):

phenytoin, barbituates, tegretol,sulfonamides, PCN, steroids
http://images.google.com/imgres?imgurl=http://www.bioon.com/figure/UploadFiles/200706/20076122818459.jpg&imgrefurl=http://www.bioon.com/figure/200706/297412.shtml&usg=__W8VsBdOeIjmTOJtlwEDnt_8_OHA=&h=354&w=500&sz=36&hl=en&start=54&um=1&itbs=1&tbnid=jGWXkPS4kIZ-yM:&tbnh=92&tbnw=130&prev=/images%3Fq%3Dtoxic%2Bepidermal%2Bnecrolysis%26ndsp%3D18%26hl%3Den%26sa%3DN%26start%3D36%26um%3D1


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TEN-contd


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TEN- contd. SCORTEN

A score of 0-1 indicates a mortality risk of 3.2%; score of 2,12.1%; score of 3, 35.3%; score of 4, 58.3%; and a score of 5

or more, 90%. Each of the following independent prognosticfactors is given a score of one:

Age older than 40 years

Heart rate of greater than 120 beats per minute

Cancer/hematologic malignancy

Involved body surface area of greater than 10% Serum urea level of more than 10 mmol/L

Serum bicarbonate level of less than 20 mmol/L

Serum glucose level of more than 14 mmol/L


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Mechanism of TENS

Delayed Hypersensitivity

Antigen native drug Accumulation of interstitial fluid under

necrotic epidermis; T lymphocytes that areable to kill autologous lymphocytes and

keratinocytes in a drug specific, HLA-restricted mediated pathway

Epidermis overexpresses TNF-alpha

stimulates cytotoxic T lymphocytes

A o tosis
http://missinglink.ucsf.edu/lm/DermatologyGlossary/img/Dermatology%20Glossary/Glossary%20Histo%20Images/EM-like_Toxic_epidermal_necrolysis-212.jpg


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Tegretol and TEN

Strongly associated with HLA-B*1502

Commonly reaction seen within two monthsof drug initiation

However can be seen in long-term use


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Steven-Johnson Syndrome

Widespread dusky macules and mucosal

involvement Due to drugs

Limited to < 10% of BSA

SJS/TENs overlap 10-30% BSA

TEN > 30% BSA
http://missinglink.ucsf.edu/lm/DermatologyGlossary/img/Dermatology%20Glossary/Glossary%20Clinical%20Images/Stevens_Johnson-28.jpg


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SJS and TEN

Acute symptoms, painful skin lesions, fever > 39, pharyngitis,visual impairment

Mortality 10-30% No treatment of proven efficacy

Early diagnosis, immediate discontinuation of any offendingdrug

No RCT exist but IVIG is second line

G-CSF if leukopenia exists (again no data) Early retrospective studies suggested that corticosteroids

increased hospital stays and complication rates.


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Erythema Multiforme

Dusky violet color or petechiae in the center of the lesions Target or iris lesions Symmetric on palms, soles, knees, elbows Mycoplasma, HSV, idiopathic, rarely drugs; PCN, sulfa, phenytoin May involve of mucous membranes, Hemorrhagic crusts of the lips

(SJS, HSV, PV, Paraenoplastic) Fever, malaise, myalgias, sore throat, and cough may accompany the

eruption

Resolve over 3-6 weeks but may recur Can follow vaccinations, XRT, exposure to environmental toxins
http://images.google.com/imgres?imgurl=http://www.aic.cuhk.edu.hk/web8/0024_erythema_multiforme.JPG&imgrefurl=http://www.aic.cuhk.edu.hk/web8/erythema_multiforme.htm&usg=__jXPXF7U-xBpv7638qfemTY4o2po=&h=318&w=452&sz=10&hl=en&start=2&um=1&itbs=1&tbnid=bJGKrQ5in6F2_M:&tbnh=89&tbnw=127&prev=/images%3Fq%3Derythema%2Bmultiforme%26hl%3Den%26um%3D1


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Drug Rash with Eosinophilia and

Systemic Symptoms (DRESS)

Widespread erythematous eruption

Fever, facial/periorbital edema, tender generalizedlymphadenopathy (atypical lymphocytes andeosinophils), leukocytosis, hepatitis, nephritis,pneumonitis

Eruption recur with re-challenge

Onset 2-8 weeks after drug is started and lastslonger

Mortality 10%


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Staphylococcal Scalded Skin

Syndrome (SSSS)

Redness or tenderness of the face, trunk, intertriginous zones

Short lived flaccid bullae and a slough of superficial epidermis

Crusted areas develop around the mouth Distinguishing features: young age group (infants), more

superficial, no oral lesions, shorter course

Associated with Staph exfoliative toxin

Lesions are sterile vs bullous impetigo

Conjuctivitis, rhinorrhea, Otitis media, pharyngitis


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SSSS


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Porphyria Cutanea Tarda

Sun exposed areas mainly hands and face

Skin is fragile which leads to tense vesicles => milia=> epidermoid inclusion cysts

Hypertrichosis

Porphyria Variegata: PCT + systemic findings

Drug-induced psuedoporphyria: Naproxen, Lasix,tetracycline, Tegretol is porphyrinogenic

Attacks can be precipitated by infections, surgery,ETOH


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Blistering Metabolic Disorders

Comatose patients and decreased

cutaneous blood flow; pressure points Diabetes Mellitus; distal extremities
http://dermatology.cdlib.org/DOJvol1num2/diabetes/diabetes-images/D10074m.jpg


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References

Harrisons Internal Medicine 17 th ed.

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2.15.10 Tichy Blistering Skin Diseases