Dermatitis, Exfoliative

Essentials of Diagnosis

• Scaling and erythema over most of the body

• Itching, malaise, fever, chills, weight loss

General Considerations

• A preexisting dermatosis is the cause in up to two-thirds of cases, including

  • Psoriasis

  • Atopic dermatitis

  • Contact dermatitis

  • Pityriasis rubra pilaris

  • Seborrheic dermatitis

• Other causes

  • Reactions to topical or systemic drugs (accounts for about 15% of cases)

  • Cancer (paraneoplastic symptom of lymphoma, solid tumors and, most commonly, cutaneous T cell lymphoma) accounts for 10%

  • Idiopathic in ~10%

• At the time of acute presentation, without a clear-cut prior history of skin disease or drug exposure, it may be impossible to make a specific diagnosis of the underlying condition, and diagnosis may require continued observation

Etiology

• Idiopathic

• Drug eruption (eg, proton pump inhibitors, nevirapine)

• Seborrheic dermatitis

• Contact dermatitis

• Atopic dermatitis

• Psoriasis

• Cancer (Sézary syndrome of cutaneous T cell lymphoma, Hodgkin disease)

• Pityriasis rubra pilaris

Symptoms and Signs

• Symptoms may include itching, weakness, malaise, fever, and weight loss

• Chills are prominent

• Redness and scaling is widespread

• Loss of hair and nails can occur

• Generalized lymphadenopathy may be due to lymphoma or leukemia or may be reactive

• The mucosae are spared

Differential Diagnosis

• Psoriasis

• Seborrheic dermatitis

• Drug eruption

• Toxic shock syndrome (staphylococcal or streptococcal)

• Scarlet fever

• Staphylococcal scalded skin syndrome

• Erythema multiforme or toxic epidermal necrolysis

Laboratory Tests

• Peripheral leukocytes may show clonal rearrangements of the T cell receptor in Sézary syndrome

Diagnostic Procedures

• A skin biopsy is required and may show changes of a specific inflammatory dermatitis or an underlying cutaneous T cell lymphoma or Sézary syndrome

Medications

• See Table 6–2

• Stop all drugs, if possible

• Systemic corticosteroids may provide remarkable improvement in severe or fulminant exfoliative dermatitis, but long-term therapy should be avoided

• For cases of psoriatic erythroderma and pityriasis rubra pilaris, either acitretin, methotrexate, cyclosporine, or a tumor necrosis factor (TNF) inhibitor may be indicated

• Erythroderma secondary to lymphoma or Sézary syndrome requires specific topical or systemic chemotherapy

• Suitable antibiotic drugs with coverage for Staphylococcus should be given when there is evidence of bacterial infection

Therapeutic Procedures

• Home treatment is with cool to tepid baths and application of mid-potency corticosteroids under wet dressings or with the use of an occlusive plastic suit

Follow-Up

• It may be impossible to identify the cause of exfoliative dermatitis early in the course of the disease, so careful follow-up is necessary

Complications

• Debility (protein loss) and dehydration may develop in patients with generalized inflammatory exfoliative erythroderma

• Sepsis

• Systemic corticosteroids must be used with caution because some patients with erythroderma have psoriasis and could develop pustular flare

Prognosis

• Most patients recover completely or improve greatly over time but may require long-term therapy

• Deaths are rare in the absence of cutaneous T cell lymphoma

• A minority of patients will suffer from undiminished erythroderma for indefinite periods

When to Refer

• Early referral to dermatology is frequently helpful

• Diagnosis of cutaneous T cell lymphoma and/or Sézary syndrome should prompt referral to oncology

When to Admit

• If the exfoliative erythroderma becomes chronic and is not manageable in an outpatient setting

• Keep the room at a constant warm temperature and provide the same topical treatment as for an outpatient