Exfoliative erythroderma

Background

Normal dermal anatomy.

• Also known as exfoliative dermatitis
• Diffuse, widespread scaly dermatitis that covers most of body surface
• Cutaneous reaction to a drug or chemical agent or underlying systemic or cutaneous disease
• Males affected twice as often as females
• Most patients >40 years old

Clinical Features

Erythroderma

• Generalized erythema, warmth, scaling
• Can be pruritic and painful
• Abrupt onset if related to drug, contact allergen, or malignancy; gradual onset if related to underlying cutaneous disorder
• Generally starts on face and trunk with progression to other skin surfaces
• Low-grade fever common
• Tends to be a chronic condition, mean duration 5 years

Differential Diagnosis

• Erythema multiforme
• Stevens-Johnson syndrome and toxic epidermal necrolysis
• Drug rash
• DRESS syndrome

Erythematous rash

• Positive Nikolsky’s sign
  • Febrile
    • Staphylococcal scalded skin syndrome (children)
    • Toxic epidermal necrolysis/SJS (adults)
  • Afebrile
    • Toxic epidermal necrolysis
• Negative Nikolsky’s sign
  • Febrile
    • Toxic shock syndrome
    • Kawasaki disease
    • Scarlet fever
    • DRESS syndrome
    • Cellulitis
    • Acute generalized exanthematous pustulosis
  • Afebrile
    • Alcohol intoxication
    • Anaphylaxis
    • Cellulitis
    • Dermatitis (multiple types)
    • Drug rash
    • Erysipelas
    • Erythema multiforme
    • Exfoliative erythroderma
    • Scombroid
    • Vancomycin infusion reaction

Evaluation

Workup

• CBC, CMP, ESR
• CXR
• Determine underlying cause, including evaluation for underlying malignancy and biopsy of skin

Table of Severe Drug Rashes

Charateristic	DRESS	SJS/TEN	AGEP	Erythroderma
Image
Onset of eruption	2-6 weeks	1-3 weeks	48 hours	1-3 weeks
Duration of eruption (weeks)	Several	1-3	<1	Several
Fever	+++	+++	+++	+++
Mucocutaneous features	Facial edema, morbilliform eruption, pustules, exfoliative dermattiis, tense bullae, possible target lesions	Bullae, atypical target lesions, mucocutaneous erosions	Facial edema, pustules, tense bullae, possible target lesions, possibl emucosal involvement	Erythematous plaques and edema affecting >90% of total skin surface with or without diffuse exfoliation
Lymph node enlargement	+++	-	+	+
Neutrophils	Elevated	Decreased	Very elevated	Elevated
Eosinophils	Very elevated	No change	Elevated	Elevated
Atypical lymphocytes	+	-	-	+
Hepatitis	+++	++	++	-
Other organ involvement	Interstitial nephritis, pneumonitis, myocarditis, and thydoiditis	Tubular nephritis and tracheobronical necrosis	Possible	Possible
Histological pattern of skin	Perivascular lymphocytcic infiltrate	Epidermal necrosis	Subcorneal pustules	Nonspecific, unless reflecting Sezary syndrome or other lymphoma
Lymph node histology	Lymphoid hyperplasia	-	-	No, unless reflecting Sezary syndrome or other malignancy
Mortality (%)	10	5-35	5	5-15

Management

• Emergent dermatology consult
• Fluid replacement for hypovolemia, monitor fluid intake
• Warming measures for hypothermia
• Wound care
• Discontinue all unnecessary medications
• Systemic corticosteroids after dermatology consult
• Antibiotics if evidence of secondary infection

Disposition

• Admit

Complications

• Hypothermia
• fluid/electrolyte/protein loss
• Invasion of bacteria and opportunistic organisms through the skin
• High-output heart failure due to vasodilatation

See Also

• Erythematous rash

References

• Tintinalli's Emergency Medicine 7th Edition, pg 1614, 1617
• Harwood-Nuss' Clinical Practice of Emergency Medicine 6th Edition, pg 821