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Blistering Skin Eruptions

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

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Blistering Skin Eruptions

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  1. Blistering Skin Eruptions Jill Tichy, PGY III February 15th, 2010

  2. 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

  3. 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)

  4. 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

  5. TEN-cont’d

  6. 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

  7. 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

  8. 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

  9. 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

  10. 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.

  11. 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

  12. 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%

  13. 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

  14. SSSS

  15. 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

  16. Blistering Metabolic Disorders • Comatose patients and decreased cutaneous blood flow; pressure points • Diabetes Mellitus; distal extremities

  17. References • Harrison’s Internal Medicine 17 th ed. • Google Images

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