Transcript Slide 1

Blistering Skin Eruptions
Jill Tichy, PGY III
February 15th, 2010
Causes of Vesicles/Bullae
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Primary Cutaneous Disease: Pemphigus, Bullous
Pemphigus, Dermatitis Herpatiformis, Contact
Dermatitis, Erythema Multiforme, Stevens-Johnson
syndrome, Toxic Epidermal Necrolysis, VZV, HSZ,
Hand-foot-and-mouth disease, Staphylococcal
scalded-skin syndrome, Scarlet Fever, Toxic Shock
Syndrome, Exfoliative Erythroderma Syndrome
Systemic Diseases: Paraneoplastic pemphigus,
Porphyria Cutanea Tarda, Porphyria Variegata
Nikolsky’s Sign
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Staphylococcal Scalded Skin Syndrome
SJS/TENS
Positive when slight rubbing of the skin results in
exfoliation of the skin's outermost layer
A "positive" Nikolsky's sign is associated with
pemphigus vulgaris.
Nikolsky's sign is useful in differentiating between
pemphigus vulgaris (where it is present or positive)
and bullous pemphigoid (where it is absent)
Toxic Epidermal Necrolysis
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Bullae that arise on the widespread areas of
erythema 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
TEN-cont’d
TEN- cont’d. SCORTEN
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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 prognostic
factors 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
Mechanism of TENS
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Delayed Hypersensitivity
Antigen native drug
Accumulation of interstitial fluid under
necrotic epidermis; T lymphocytes that are
able to kill autologous lymphocytes and
keratinocytes in a drug specific, HLArestricted mediated pathway
Epidermis overexpresses TNF-alpha 
stimulates cytotoxic T lymphocytes 
Apoptosis
Tegretol and TEN
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Strongly associated with HLA-B*1502
Commonly reaction seen within two months
of drug initiation
However can be seen in long-term use
Steven-Johnson Syndrome
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Widespread dusky macules and mucosal
involvement
Due to drugs
Limited to < 10% of BSA
SJS/TENs overlap 10-30% BSA
TEN > 30% BSA
SJS and TEN
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Acute symptoms, painful skin lesions, fever > 39, pharyngitis,
visual impairment
Mortality 10-30%
No treatment of proven efficacy
Early diagnosis, immediate discontinuation of any offending
drug
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.
Erythema Multiforme
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“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
Drug Rash with Eosinophilia and
Systemic Symptoms (DRESS)
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Widespread erythematous eruption
Fever, facial/periorbital edema, tender generalized
lymphadenopathy (atypical lymphocytes and
eosinophils), leukocytosis, hepatitis, nephritis,
pneumonitis
Eruption recur with re-challenge
Onset 2-8 weeks after drug is started and lasts
longer
Mortality 10%
Staphylococcal Scalded Skin
Syndrome (SSSS)
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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
SSSS
Porphyria Cutanea Tarda
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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
Blistering Metabolic Disorders
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Comatose patients and decreased
cutaneous blood flow; pressure points
Diabetes Mellitus; distal extremities
References
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Harrison’s Internal Medicine 17 th ed.
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