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Blistering Skin Eruptions. Jill Tichy, PGY III February 15 th , 2010. Causes of Vesicles/Bullae.

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blistering skin eruptions

Blistering Skin Eruptions

Jill Tichy, PGY III

February 15th, 2010

causes of vesicles bullae
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
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
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 scorten
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
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
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
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
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
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
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
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
porphyria cutanea tarda
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
Blistering Metabolic Disorders
  • Comatose patients and decreased cutaneous blood flow; pressure points
  • Diabetes Mellitus; distal extremities
references
References
  • Harrison’s Internal Medicine 17 th ed.
  • Google Images
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