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October 2004

Generalized Exfoliative Erythroderma Since Birth—Quiz Case

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Arch Dermatol. 2004;140(10):1275-1280. doi:10.1001/archderm.140.10.1275-a

A 29-year-old man presented with generalized erythroderma and scaling. Physical examination revealed erythema with lamellar ichthyotic scales over the entire body and focal erosions on the back (Figure 1 and Figure 2). Although growth of scalp hairs was abundant, individual hairs appeared to be brittle. The skin condition had been present since birth, and the patient reported episodic erythrodermic flares with scaling. Examination of his teeth and nails revealed no abnormalities. His family history was noncontributory, with the exception of a distant relative who had an unspecified skin condition. Laboratory tests showed pronounced elevation of IgE levels (>2000 kU/L; reference range, <100 kU/L), with specific IgE reagins to various airborne and food allergens. A routine histopathologic section of erythrodermic skin is shown in Figure 3, and a microscopic photograph of a hair shaft is shown in Figure 4.