Erythroderma

Aka: Erythroderma, Exfoliative Erythroderma Syndrome, Exfoliative Dermatitis

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II. Definitions

  1. Erythroderma (Exfoliative Dermatitis)
    1. Serious to life-threatening dermatosis with generalized skin erythema and Scaling >80% surface area

III. Epidemiology

  1. Age over 40-50 years old most common
  2. More common in males

IV. Pathophysiology

  1. Generalized cutaneous vasodilation with inflammatory cell leakage into Dermis
  2. Generalized Edema and inflammation ensues
  3. Scaling follows inflammation after 5 days
  4. Rapid cell turnover results in frequent loss of cell contents including Proteins

V. Symptoms

  1. Diffuse Pruritus
  2. Constitutional symptoms (generalized weakness, malaise, chills)

VI. Signs

  1. Ill or toxic appearance
  2. Diffuse, generalized bright erythematous skin (typically including palms and soles)
  3. Diffuse Scaling of skin (within 5 days of onset)
  4. Onycholysis
  5. Alopecia

VII. Causes

  1. Preexisting dermatosis in 50% of cases (typically more gradual onset)
    1. Psoriasis
      1. Most common Erythroderma cause in adults (esp. with Medication Withdrawal)
      2. Central face is typically spared
      3. Pre-existing psoriatic Plaques may be obscured by diffuse exfoliation
        1. Plaques may also be replaced by sterile subcorneal Pustules
      4. Observe for nail changes (Onycholysis, Nail Pitting, subungual hyperkeratosis)
    2. Atopic Dermatitis (Eczema)
      1. Widespread erythematous dermatitis with intense Pruritus
      2. Lichenification with white scale in regions of chronic scratching
    3. Seborrheic Dermatitis
    4. Pityriasis rubra pilaris
      1. Salmon or orange-red colored skin lesions with variable Scaling and islands of sparing
      2. Follicular keratotic Papules on elbows and knees, as well as dorsal fingers
    5. Ichthyosis
    6. Lichen Planus
  2. Drug Reaction (rapid onset)
    1. See Life-Threatening Drug-Induced Rashes
    2. See Fixed Drug Eruption
    3. See DRESS Syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms)
    4. Typical onset as as Morbilliform or Scarlatiniform Rash
    5. Fever, Lymphadenopathy and hepatitis may be present
    6. Most common causes
      1. Antibiotics
      2. Seizure medications
      3. Cardiac medications
  3. Infection
    1. HIV Infection
    2. Toxic Shock Syndrome
    3. Norwegian crusted Scabies
    4. Staphylococcal Scalded Skin Syndrome
      1. Seen in infants and young children within 24 to 48 hours of Staphylococcus aureus infection
      2. Painful diffuse erythema, followed by flaccid subcorneal bullae that slough with raw, red exposed skin
      3. Spares mucous membranes
  4. Leukemia or Lymphoma
    1. Cutaneous T-Cell Lymphoma (esp. stage 4, Mycosis Fungoides)
  5. Other Causes
    1. Stevens-Johnson Syndrome (Erythema Multiforme Major)
    2. Toxic Epidermal Necrolysis

VIII. Management

  1. Consult regional burn unit
  2. Hospitalize all patients with suspected Erythroderma
  3. Treat underlying cause if identified (e.g. Toxic Shock Syndrome)
  4. Manage complications (similar to Burn Injury)
    1. Dehydration
    2. Electrolyte replacement
    3. Secondary infections
  5. Skin care - layered approach
    1. Layer 1: Low to moderate potency Corticosteroid (e.g. Triamcinolone)
    2. Layer 2: Moist wrap or clothes
    3. Layer 3: Dry layer

IX. Complications

  1. Dehydration
  2. Metabolic abnormalities
  3. High output Heart Failure
  4. Secondary Skin Infections
  5. Hypoalbuminemia
  6. Peripheral Edema
  7. Anemia
  8. Temperature instability (Hypothermia, hyperthermia)

X. References

  1. Jhun, Grock and DeClerck in Herbert (2017) EM:Rap 17(3):18-9
  2. Fitzpatrick (1992) Color Atlas of Dermatology, p. 442-7
  3. Moon (2022) Am Fam Physician 105(1): 75-6 [PubMed]

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