AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Background

Exfoliative dermatitis, or erythroderma, is an erythematous, scaly dermatitis involving most, if not all, of the skin. This generalized scaling eruption of the skin is drug induced, idiopathic, or secondary to underlying cutaneous or systemic disease.

Appreciation for this condition requires an understanding of the skin's normal epithelial layer. Normal epidermis has a continual turnover of epithelial cells. Cell division occurs near the basal layer. As cells move toward the periphery, they become well keratinized. This process requires approximately 10-12 days. Cells subsequently remain in the stratum corneum for another 12-14 days prior to being sloughed off.

In exfoliative dermatitis, the mitotic rate in the basal layer increases and overall transit time decreases; therefore, more cells are lost from the surface. The mechanism responsible for this is not known, although an immunologic basis has been suggested.

Pathophysiology

Exfoliative dermatitis may occur in response to drug therapy, systemic disease, or an idiopathic entity. As many as 40% of cases involve preexisting cutaneous disease. Approximately 10% of cases are the result of drug reactions. As many as 40% are caused by underlying systemic disease. The remaining cases are idiopathic.

Histopathologic patterns observed for drug-induced and idiopathic causes of exfoliative dermatitis are nonspecific. Biopsy findings in individuals with preexisting cutaneous or systemic disease during an exfoliative stage may reveal, inconsistently, the underlying skin lesion or pathology. Through multiple-biopsy histologic analysis, the diagnosis may be confirmed in as many as 45% of patients.

Frequency

United States

An estimated 1% of hospitalizations are for skin disease.

International

The ratio of hospitalized patients experiencing adverse drug reactions is 3 in 1000. According to one large Finnish study, approximately 1% of these instances involve exfoliative dermatitis.

Mortality/Morbidity

The mortality rate approaches 30%. In a report of 108 patients with exfoliative dermatitis who were autopsied, 87 died from the underlying disease. No cause other than exfoliation was found for the remaining 17 patients.

Race

Exfoliative dermatitis occurs in all races. In the young black male population, research suggests exfoliative dermatitis may be a marker for HIV infection.

Sex

The male-to-female ratio is 2:1.

Age

Individuals older than 40 years are affected most frequently.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

History

• Prior dermatologic illnesses
• Treatment with new medications
• Underlying systemic diseases
• • Swelling or adenopathy
  • Recurrent infections
  • Cough or change in bowel or urinary habits
• Constitutional symptoms of low-grade fever, chills, and malaise (more commonly, patients complain of skin erythema, tightness, and scaling)

Physical

• Gynecomastia is a common finding in almost all patients with exfoliative dermatitis of several weeks' duration; it is believed to be secondary to hyperestrogenism, but the precise mechanism is unknown.
• Additional findings include alopecia, dystrophic nails, and hypopigmentation and/or hyperpigmentation.
• More than 40% of patients were febrile, with temperatures higher than 38°C; a relative tachycardia also was noted.
• Firm, rubbery lymphadenopathy, referred to as dermatopathic lymphadenitis, is a common finding.
• Hepatomegaly was noted in more than 50% of patients in one series, and splenomegaly was noted in approximately 30% of patients, all of whom had lymphoma.
• Steatorrhea may develop and tends to resolve when exfoliative dermatitis clears.
• Patients with exfoliative dermatitis have increased cutaneous blood flow, transcutaneous fluid losses, and radiation and convective heat losses.
• • A number of cases of hypothermia have been reported.
  • Fluid shifts increase cardiac output, causing dyspnea, dependent edema, and cardiac failure (possibly) in some patients.
• Prostate or thyroid glands may be enlarged or nodular.

Causes

• Systemic diseases (10-40%)
• • Lymphoma - Primarily cutaneous T-cell lymphoma (CTCL)
  • Leukemia
  • Multiple myeloma
  • Carcinoma of the lung, prostate, colon, and thyroid
  • Graft versus host disease
  • Immunodeficiency, including HIV
  • Hodgkin disease
• Cutaneous diseases (10-40%)
• • Psoriasis
  • Seborrheic dermatitis
  • Atopic dermatitis
  • Stasis dermatitis
  • Contact dermatitis
  • Pityriasis rubra pilaris
  • Pemphigus foliaceus
  • Mycosis fungoides
  • Dermatophytosis
  • Lichen planus
• Drugs (3-10%)
• • Dimercaprol (British antilewisite [BAL])
  • Codeine
  • Captopril
  • Diphenylhydantoin
  • Gold
  • Iodine
  • Antimicrobials - Sulfas, penicillin (PCN), cephalosporins, minocycline, isoniazid
  • Granulocyte colony-stimulating factor (GCSF)
  • Phenytoin
  • Allopurinol
  • Mercury
  • Arsenic
  • Quinidine
  • Barbiturates
  • Trimethadione
  • Aspirin
  • Carbamazepine
• Idiopathic (15-45%)

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Lab Studies

• Perform laboratory studies based on clinical suspicion or history that may help to establish the primary cause of exfoliative dermatitis. Other laboratory studies include the following:
• • Evaluation for cardiac failure and renal and intestinal dysfunction
  • CBC with differential, liver enzymes level, creatinine level, and urinalysis
  • Serum albumin level
  • Erythrocyte sedimentation rate (ESR)
• The most common laboratory abnormalities are anemia, hypoalbuminemia, eosinophilia, and an elevated sedimentation rate.

Imaging Studies

• Obtain imaging studies based on suspicion of underlying systemic disease or when other causes are excluded.

Procedures

• Biopsy
• • Perform skin biopsy and lymph node biopsy if significant lymphadenopathy is noted.
  • Histologic findings generally are nonspecific and include hyperkeratosis, parakeratosis, acanthosis of the epidermis, and perivascular infiltrate consisting of histocytes, lymphocytes, and some eosinophils.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Emergency Department Care

Hospitalize most patients with acute exfoliative dermatitis in the intensive care unit or burn center for supportive care, fluid replacement, laboratory studies, and contact isolation for protection against secondary bacterial and fungal infections. No studies have suggested a better outcome for patients treated in burn centers.

• General measures
• • Withdraw implicated medications or treatment of identified underlying infection, disease, or both.
  • Protect patient from development of hypothermia.
  • Prescribe cool oatmeal baths.
  • Advise local moisturizing ointments, lotions, or both.
  • Advise a high-protein diet with folic acid supplementation, since protein losses may be increased as much as 30% above normal.

Consultations

Urgent consultation with a dermatologist is recommended.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Treatment of the underlying illness is key since exfoliative dermatitis resists treatment until the basic disease is treated. Frequent tub baths with emollients are indicated to provide symptomatic relief.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. Also used to treat idiopathic and acquired autoimmune disorders.

Drug Category: Antihistamines

These agents are used to treat minor allergic reactions and anaphylaxis. They are used for relief of pruritus.

Drug Category: Immunosuppressives

These agents interfere in the immune processes that promote inflammation. They are used to relieve chronic severe dermatitis.

Drug Category: Antirheumatic, Disease Modifying

These agents modulate inflammatory and immune responses.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Complications

• Bacterial or fungal superinfection
• Hypothermia
• Dehydration, electrolyte imbalance, or both
• Heart failure

Prognosis

• In general, long-term prognosis is good for patients with drug-induced disease after the offending agent is withdrawn and proper supportive measures are undertaken.
• For patients with idiopathic exfoliative dermatitis, the prognosis is poor. Frequent recurrences or chronic symptoms require long-term steroid therapy and its attendant sequelae.
• For patients with underlying disease or malignancy, prognosis rests on the outcome and course of the disease process.

Patient Education

• Avoid known etiologic agents.
• Educate patients with underlying disease about symptomatic treatment and advise that many cases spontaneously remit.
• Advise patients on protection from hypothermia.
• Advise patients to follow a high-protein diet as symptoms persist.
• Encourage patients to be diligent in watching for signs of infection.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Special Concerns

• In neonates, thoroughly investigate severe immunodeficiency as a possibility.
• Elderly patients are at risk of cardiac complications. Fluid losses and shifts often are significant, and decreased compensatory mechanisms in members of the elderly population make them particularly vulnerable.
• Use corticosteroids with caution. This syndrome has a clinically similar appearance to that of toxic epidermal necrolysis for which steroids have shown no benefit or increased morbidity or mortality.

ACKNOWLEDGMENTS

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Jonathan R Pilcher, MD, and Selwyn Waterton, MD, to the development and writing of this article.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

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Article Last Updated: Jan 15, 2008