Exfoliative Dermatitis

Exfoliative dermatitis (ED), known as erythroderma in the United Kingdom and l'homme rouge in France, refers to a total, or almost total, redness or scaling of the skin.
It is an uncommon disorder seen more often in male patients; 50 years is the average age of occurrence.
In children, ED most often is secondary to severe atopic dermatitis.
In adults, psoriasis is the most frequently associated skin disease (see Chapter 14: Psoriasis).
ED may appear suddenly or gradually, occasionally accompanied by fever, chills, and lymphadenopathy.
Less commonly, ED has been reported as a finding in the following skin disorders:
- Allergic contact dermatitis.
- Stasis dermatitis with secondary autoeczematization.
- Papulosquamous dermatitis of acquired immunodeficiency syndrome.
- Graft-versus-host disease.
- Seborrheic dermatitis (Leiner disease) in infants.
- Pemphigus foliaceus.
- Pityriasis rubra pilaris (a rare disorder of keratinization).
ED may occur as a reaction to the following drugs: sulfonamides; penicillins; antimalarials; lithium; phenothiazines; ED may be a stage in the natural history of severe eczematous dermatitis or psoriasis. Barbiturates; gold; allopurinol; NSAIDs, including aspirin; captopril; codeine; and phenytoin.
It also may be a complication or presenting symptom of the following malignant diseases:
- Mycosis fungoides (cutaneous T-cell lymphoma).
- Sézary syndrome (leukemic variant of mycosis fungoides).
- Hodgkin disease.
- Non-Hodgkin lymphoma and leukemia.
It is an idiopathic phenomenon in 20% to 30% of cases without any preceding dermatosis or systemic disease.

Clinical Manifestations ⬆ ⬇

Unless patients have a known preexisting skin condition or concurrent physical evidence of a skin disease such as psoriasis, the clinical appearance and symptoms of most cases of ED tend to be similar, consisting of the following:
- ED usually begins in a limited area; however, it may rapidly become generalized.
- Pruritus may develop and may be severe.
- Marked generalized erythema is followed by scaling (Figs. 34.46 and 34.47).
- There is edema and increased warmth of the skin.
- Lymphadenopathy, usually a reactive type (dermatopathic lymphadenopathy), is often present.
- Unlike toxic epidermal necrolysis, ED spares the mucous membranes.
- Thermoregulatory disturbances are manifested by fever or, more frequently, by hypothermia. If widespread inflammation occurs, the barrier efficiency of the skin may be impaired secondary to extensive vasodilatation.
Protein loss secondary to a massive shedding of scale may occur, with resultant hypoalbuminemia.
Rarely, high-output cardiac failure may develop, particularly in patients with a history of cardiac disease.
The following chronic changes may also be seen:
- Scaling of palms and soles (keratoderma).
- Thickening and lichenification of the skin.
- Scalp involvement, occasionally producing nonscarring alopecia.
- Nail dystrophy, onycholysis (separation of the nail plate from the nail bed), or nail shedding.
- Pigmentary changes (postinflammatory hypopigmentation or hyperpigmentation).
- Persistent generalized erythema.
- Conjunctivitis, keratitis, or ectropion.

Diagnosis ⬆ ⬇

The diagnosis of ED is made on a clinical basis and the underlying cause is often elusive.
Clinical findings, such as the characteristic lichenification and crusting of atopic dermatitis or nail pitting that suggests psoriasis, may be found.
Eliciting a history of drug ingestion or a preexisting dermatosis may be valuable.
Laboratory testing can provide serologic evidence of Sézary syndrome or leukemia.
Patch testing during a period of remission may uncover a contact allergen.

Other Information ⬆

Laboratory Evaluation

The following are possible positive laboratory findings:

Anemia (usually the anemia of chronic disease).
Decreased serum levels of protein and albumin.
Leukocytosis.
Eosinophilia.
Elevated sedimentation rate.
Elevated immunoglobulin E level (possibly supporting the diagnosis of atopic dermatitis).
Leukemia (noted on peripheral blood smear).
Imaging studies with computed tomography or magnetic resonance imaging if lymphoma or Hodgkin disease is suspected.

Differential Diagnosis

Toxic Epidermal Necrolysis (see Chapter 26: Adverse Cutaneous Drug Eruptions)

A potentially fatal condition that involves the skin and mucous membranes.
Marked erythema is quickly followed by sloughing of the skin.
Often the result of a severe drug reaction.

Management

Treatment is directed toward the underlying cause, if it is known. For example, suspected etiologic drugs or contactants should be eliminated.
Bed rest, cool compresses, lubrication with emollients, antipruritic therapy with oral antihistamines, and low- to intermediate-strength topical steroids are used.
Systemic antibiotics can be administered if signs of secondary infection are observed
In severe cases, patients frequently require hospitalization, where measures such as fluid replacement, temperature control, expert topical skin care, and systemic corticosteroids may be used.
Isotretinoin (Accutane) has been used when pityriasis rubra pilaris is the underlying cause

Exfoliative Dermatitis Secondary to Psoriasis

Possible precipitating factors (e.g., ultraviolet exposure) or drugs that are suspected to provoke ED (e.g., antimalarials) should be avoided.
Systemic and topical steroids are helpful, except that they may worsen psoriasis and have been known to precipitate ED or an acute fulminant form of pustular psoriasis, known as pustular psoriasis of Von Zumbusch. This worsening of psoriasis tends to occur after steroid withdrawal.
If conservative therapy fails, methotrexate, cyclosporine, and retinoids (e.g., acitretin) are additional therapeutic options.
Phototherapy, photopheresis, and photochemotherapy, as well as monoclonal antibodies such as infliximab(Remicade) and Adalimumab (Humira), may be effective.
For a further discussion of psoriasis (see Chapter 14: Psoriasis).

Prognosis

The course of ED depends on its underlying origin.
ED resulting from a drug eruption may clear in days to weeks, after the drug is stopped.
In some cases, ED may persist for many years, with exacerbations and remissions with no apparent diagnosis.
In patients with an identified underlying cause, the course and prognosis generally parallel the primary disease.
Acute, severe episodes, particularly in elderly persons or in persons with preexisting heart disease, have a more guarded prognosis.
In patients with idiopathic ED, the prognosis is poor, and recurrences are not uncommon.

Points to Remember

In its more severe manifestations, ED is a medical and dermatologic emergency. Consultation and ongoing management, using the expertise of both disciplines, are often necessary.
In many cases, the underlying cause is never established.

section name header

Basics ⬇

Clinical Manifestations ⬆ ⬇

Diagnosis ⬆ ⬇

Other Information ⬆