Blistering Skin Eruptions

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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, 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

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Nikolskys 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)

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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

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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 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

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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

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Tegretol and TEN

• Strongly associated with HLA-B1502
• Commonly reaction seen within two months of 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 lt 10 of BSA
• SJS/TENs overlap 10-30 BSA
• TEN gt 30 BSA

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SJS and TEN

• Acute symptoms, painful skin lesions, fever gt 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.

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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

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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

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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 gt
  milia gt 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

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References

• Harrisons Internal Medicine 17 th ed.
• Google Images