blistering skin eruptions jill tichy, pgy iii february 15 th, 2010
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Blistering Skin Eruptions
Jill Tichy, PGY III
February 15th, 2010
Causes of Vesicles/Bullae
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
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
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
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
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, HLA-restricted mediated pathway
Epidermis overexpresses TNF-alpha stimulates cytotoxic T lymphocytes Apoptosis
Tegretol and TEN
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
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
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
“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)
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)
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
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
Comatose patients and decreased cutaneous blood flow; pressure points
Diabetes Mellitus; distal extremities
References
Harrison’s Internal Medicine 17 th ed. Google Images
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