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Exfoliative Erythroderma Syndrome (EES)

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ICD-9: 695.9 Image not available.

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  • EES is a serious, at times life-threatening, reaction pattern of the skin characterized by a uniform redness, infiltration, and scaling involving practically the entire skin.

  • It is associated with fever, malaise, shivers, and generalized lymphadenopathy.

  • Two stages, acute and chronic, merge one into the other. In the acute and subacute phases, there is rapid onset of generalized vivid red erythema and fine branny scales; the patient feels hot and cold, shivers, and has fever. In chronic EES, the skin thickens, and scaling continues and becomes lamellar.

  • There may be loss of scalp and body hair, and the nails become thickened and separated from the nail bed (onycholysis).

  • There may be hyperpigmentation or patchy loss of pigment in patients whose normal skin color is brown or black.

  • The most frequent preexisting skin disorders are (in order of frequency) psoriasis, atopic dermatitis, adverse cutaneous drug reaction, lymphoma, allergic contact dermatitis, and pityriasis rubra pilaris.

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[See “Sézary Syndrome” in Section 21 for a special consideration of this form of EES.]

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Epidemiology

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Age of Onset
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Usually >50 years; in children, EES usually results from atopic dermatitis.

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Sex
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Males > females.

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Etiology

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Some 50% of patients have history of preexisting dermatosis. Most frequent are psoriasis, atopic dermatitis, adverse cutaneous drug reactions, cutaneous T-cell lymphoma (CTCL), allergic contact dermatitis, and pityriasis rubra pilaris (Table 8-1). Drugs most commonly implicated in EES are shown in Table 8-2. In 20% of patients, it is not possible to identify the cause.

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Table Graphic Jump Location
TABLE 8-1ETIOLOGY OF EXFOLIATIVE DERMATITIS IN ADULTS
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Table Graphic Jump Location
TABLE 8-2DRUGS THAT CAUSE EXFOLIATIVE DERMATITIS

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