para>Systemic steroids may be helpful in some cases but should be avoided in suspected cases of psoriasis.
When used for treatment, systemic steroids should be tapered slowly.
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GENERAL MEASURES
- Regardless of underlying cause, management involves maintenance of fluid and electrolyte balance, prevention of hypothermia, and treatment of pruritus and secondary infection.
- In general, outpatient management is sufficient. Hospitalization may be necessary in children, the elderly, or in patients with secondary infection, dehydration, or heart failure.
- Withdrawal of any implicated medications or treatment of any identified underlying infection/disease
- When ED evolves rapidly, the patient frequently requires hospitalization, where measures such as fluid replacement, temperature control, and expert topical skin care are available.
MEDICATION
First Line
- Midpotency topical steroids
- In addition, treatment specific to any underlying infection or disease should be provided.
- Systemic steroids: initial dosage equivalent to prednisone 40 mg/day or 1 to 2 mg/kg/day with increases in dosage by 20 mg/day if no response after 3 to 4 days. Subsequently, dosage should be slowly tapered as symptoms are controlled.
- If psoriasis is determined to be the underlying cause of the ED, acitretin, methotrexate, cyclosporine, or anti-TNF biologics are preferred over systemic steroids, which may exacerbate psoriasis (3)[B].
Second Line
- Cyclosporine: 5 mg/kg/day; taper to 1 to 3 mg/kg/day.
- Methotrexate, azathioprine, or mycophenolate mofetil
- Phototherapy or photochemotherapy also may be useful therapy for treating ED associated with mycosis fungoides.
- Oral acitretin and isotretinoin have been used when pityriasis rubra pilaris is the underlying cause.
- Antimetabolites/cytotoxic drugs
- Bexarotene
- TNF antagonists: infliximab, etanercept, adalimumab (4)
- Intravenous immunoglobulin
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
- Impending or actual heart failure
- Inability to control ED on an outpatient basis
- The very young and elderly with fulminant disease are more apt to develop complications, such as septicemia and high-output cardiac failure, and are best managed in hospital settings (5,6)[B].
Nursing
Bed rest, cool compresses, lubrication with emollients, antipruritic therapy with PO antihistamines, and low- to intermediate-strength topical steroids á
ONGOING CARE
DIET
- Increase fluid intake.
- Ensure adequate nutrition with emphasis on sufficient protein intake.
PROGNOSIS
- Prognosis of ED depends largely on underlying etiology.
- In patients with idiopathic ED, the prognosis is poor and recurrences are not uncommon.
- In patients with an identified underlying cause, the course and prognosis generally will parallel those of the primary disease. For example, in patients who have underlying psoriasis or atopic dermatitis, the progression of disease generally is gradual.
- ED due to a drug eruption usually clears within 2 to 6 weeks after the drug is discontinued.
Geriatric Considerations
Acute, severe episodes, particularly in elderly persons or in persons with preexisting heart disease, also have more guarded prognoses.
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Pediatric Considerations
A study of erythrodermic pediatric patients indicated that age <3 years, fever, ill appearance, hypotension, and elevated creatinine levels are poor prognostic signs, and the possibility of toxic shock syndrome should be considered.
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COMPLICATIONS
- Secondary infection, sepsis due to loss of effective barrier
- An impaired skin barrier with rapid keratinocyte turnover and loss and an increase in cutaneous blood perfusion may contribute to the following:
- Dehydration/electrolyte disturbances
- Hypoalbuminemia
- Edema
- Heat loss and hypothermia
- Possible high-output cardiac failure and death
- Depending on the underlying cause and possible complications, overall mortality ranges from 20% to 40%.
- Patients with idiopathic ED have a higher relapse rate and a worse prognosis.
- Complications in ED vary depending on underlying disease and response to treatment.
- Secondary infection, dehydration, electrolyte imbalance, temperature dysregulation, and high-output cardiac failure are all potential complications.
REFERENCES
11 Li áJ, Zheng áHY. Erythroderma: a clinical and prognostic study. Dermatology. 2012;225(2):154-162.22 Miyagaki áT, Sugaya áM. Erythrodermic cutaneous T-cell lymphoma: how to differentiate this rare disease from atopic dermatitis. J Dermatol Sci. 2011;64(1):1-6.33 Rosenbach áM, Hsu áS, Korman áNJ, et al. Treatment of erythrodermic psoriasis: from the medical board of the National Psoriasis Foundation. J Am Acad Dermatol. 2010;62(4):655-662.44 Zattra áE, Belloni Fortina áA, Peserico áA, et al. Erythroderma in the era of biological therapies. Eur J Dermatol. 2012;22(2):167-171.55 Byer áRL, Bachur áRG. Clinical deterioration among patients with fever and erythroderma. Pediatrics. 2006;118(6):2450-2460.66 Pruszkowski áA, Bodemer áC, Fraitag áS, et al. Neonatal and infantile erythrodermas: a retrospective study of 51 patients. Arch Dermatol. 2000;136(7):875-880.
ADDITIONAL READING
- Okoduwa áC, Lambert áWC, Schwartz áRA, et al. Erythroderma: review of a potentially life-threatening dermatosis. Indian J Dermatol. 2009;54(1):1-6.
- Rothe áMJ, Bernstein áML, Grant-Kels áJM. Life-threatening erythroderma: diagnosing and treating the "red man."Ł Clin Dermatol. 2005;23(2):206-217.
SEE ALSO
Algorithm: Rash, Focal á
CODES
ICD10
- L26 Exfoliative dermatitis
- L53.9 Erythematous condition, unspecified
- L27.0 Gen skin eruption due to drugs and meds taken internally
ICD9
- 695.89 Other specified erythematous conditions
- 695.9 Unspecified erythematous condition
- 693.0 Dermatitis due to drugs and medicines taken internally
SNOMED
- Erythroderma (disorder)
- Generalized erythroderma
- Drug-induced erythroderma (disorder)
- Generalized exfoliative dermatitis (disorder)
- Erythroderma of unknown etiology (disorder)
CLINICAL PEARLS
- In its more severe manifestations, ED is a medical and dermatologic emergency.
- In many cases, the underlying cause is never established.
- Prednisone is generally contraindicated in ED secondary to psoriasis, whereas an oral retinoid (acitretin) or an immunosuppressant, such as cyclosporine, may be an appropriate choice for this disease.