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Key Features

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 (underlying 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

Clinical Findings

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

Diagnosis

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

Treatment

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

Outcome

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 ...

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